Cited by 205Adrenal Hormones and DisordersSexual Differentiation and DisordersMetabolism and Genetic Disorders

The DOI matches its Crossref record; 266 of the 272 references it cites were found in a registry or an index.

Disorders in the initial steps of steroid hormone synthesis

Miller, Walter L.·2017·The Journal of Steroid Biochemistry and Molecular Biology

DOI 10.1016/j.jsbmb.2016.03.009

Open this paper in CoChat — free account

Abstract

Steroidogenesis begins with cellular internalization of low-density lipoprotein particles and subsequent intracellular processing of cholesterol. Disorders in these steps include Adrenoleukodystrophy, Wolman Disease and its milder variant Cholesterol Ester Storage Disease, and Niemann-Pick Type C Disease, all of which may present with adrenal insufficiency. The means by which cholesterol is directed to steroidogenic mitochondria remains incompletely understood. Once cholesterol reaches the outer mitochondrial membrane, its delivery to the inner mitochondrial membrane is regulated by the steroidogenic acute regulatory protein (StAR). Severe StAR mutations cause classic congenital lipoid adrenal hyperplasia, characterized by lipid accumulation in the adrenal, adrenal insufficiency, and disordered sexual development in 46,XY individuals. The lipoid CAH phenotype, including spontaneous puberty in 46,XX females, is explained by a two-hit model. StAR mutations that retain partial function cause a milder, non-classic disease characterized by glucocorticoid deficiency, with lesser disorders of mineralocorticoid and sex steroid synthesis. Once inside the mitochondria, cholesterol is converted to pregnenolone by the cholesterol side-chain cleavage enzyme, P450scc, encoded by the CYP11A1 gene. Rare patients with mutations of P450scc are clinically and hormonally indistinguishable from those with lipoid CAH, and may also present as milder non-classic disease. Patients with P450scc defects do not have the massive adrenal hyperplasia that characterizes lipoid CAH, but adrenal imaging may occasionally fail to distinguish these, necessitating DNA sequencing.

Integrity signals

Registry

Crossref · 10.1016/j.jsbmb.2016.03.009

Cites

266 of 266 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

The Molecular Biology, Biochemistry, and Physiology of Human Steroidogenesis and Its Disorders
Miller, W. L., & Auchus, R. J. (2011) · Endocrine Reviews · doi:10.1210/er.2010-0013 · Cited by 2,458
Self-citation
Membrane Fusion: Grappling with SNARE and SM Proteins
Südhof, T. C., & Rothman, J. E. (2009) · Science · doi:10.1126/science.1161748 · Cited by 2,027
Protein Sensors for Membrane Sterols
Goldstein, J. L., DeBose-Boyd, R. A., & Brown, M. S. (2006) · Cell · doi:10.1016/j.cell.2005.12.022 · Cited by 1,517
Structural and functional features and significance of the physical linkage between ER and mitochondria
Csordás, G., Renken, C. W., Várnai, P., Walter, L., Weaver, D., BUTTLE, K. F., Balla, T., Mannella, C. A., & Hajnóczky, G. (2006) · The Journal of Cell Biology · doi:10.1083/jcb.200604016 · Cited by 1,377
The LDL Receptor
Goldstein, J. L., & Brown, M. S. (2009) · Arteriosclerosis, Thrombosis, and Vascular Biology · doi:10.1161/atvbaha.108.179564 · Cited by 1,344
Niemann-Pick C1 Disease Gene: Homology to Mediators of Cholesterol Homeostasis
Carstea, E. D., Morris, J. A., Coleman, K. G., Loftus, S. K., Zhang, D., Cummings, C., Gu, J., Rosenfeld, M. A., Pavan, W. J., Krizman, D. B., et al. (1997) · Science · doi:10.1126/science.277.5323.228 · Cited by 1,293
An ER-Mitochondria Tethering Complex Revealed by a Synthetic Biology Screen
Kornmann, B., Currie, E., Collins, S. R., Schuldiner, M., Nunnari, J., Weissman, J. S., & Walter, P. (2009) · Science · doi:10.1126/science.1175088 · Cited by 1,275
The purification, cloning and expression of a novel luteinizing hormone-induced mitochondrial protein in MA-10 mouse Leydig tumor cells. Characterization of the steroidogenic acute regulatory protein (StAR)
Clark, B. J., Wells, J., King, S. R., & Stocco, D. M. (1994) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)46930-x · Cited by 1,168
Regulation of the acute production of steroids in steroidogenic cells
STOCCO, D. M., & CLARK, B. J. (1996) · Endocrine Reviews · doi:10.1210/edrv-17-3-221 · Cited by 1,057
Role of steroidogenic acute regulatory protein in adrenal and gonadal steroidogenesis
Lin, D., Sugawara, T., Strauss, J. F., Clark, B. J., Stocco, D. M., Saenger, P., Rogol, A., & Miller, W. L. (1995) · Science · doi:10.1126/science.7892608 · Cited by 1,030
Self-citation

Flagged first, then by citations

Show all 272 references

6 of the 272 references couldn’t be matched to a known paper.

Research works printed but not linked: 6

Tilip, 1913, Hochgradige Verfettung der Nebenniere eines Sauglings, Verhandlungen der Deutschensch Gesellschaft für Pathologie, 16, 305
Brutschy, 1920, Frunkfurter Zeitschriff für Pathologie, 24, 203
Zahn, 1948, Ueber intersexualitat und Nebennierenhyperplasie, Schweizerische Medizinische Wochcnschrift, 78, 480
Prader, 1955, Das Syndrom des Pseudohermaphroditismus masculinus bei kongenitaler Nebennierenrindenhyperplasie ohne Androgenuberproduktion (adrenaler Pseudohermaphroditismus masculinus), Helv Paed Acta, 10, 397
Prader, 1962, Zur Genetik der kongenitalen Lipoidhyperplasie der Nebennieren, Helvetica Paediatria Acta, 17, 285
Tsutsui, 1970, An autopsy case of congenital lipoid adrenal hyperplasia of the adrenal cortex, Acta Path. Japon., 20, 227

Sorted by citations

Congenital adrenal hyperplasia
El-Maouche, D., Arlt, W., & Merke, D. P. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31431-9 · Cited by 669
Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management
Claahsen - van der Grinten, H. L., Speiser, P. W., Ahmed, S. F., Arlt, W., Auchus, R. J., Falhammar, H., Flück, C. E., Guasti, L., Huebner, A., Kortmann, B. B. M., et al. (2022) · Endocrine Reviews · doi:10.1210/endrev/bnab016 · Cited by 556
Self-citation
Genetics of congenital adrenal hyperplasia
Krone, N., & Arlt, W. (2009) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2008.10.014 · Cited by 312
Steroidogenesis: Unanswered Questions
Miller, W. L. (2017) · Trends in Endocrinology & Metabolism · doi:10.1016/j.tem.2017.09.002 · Cited by 226
Self-citation
Mitochondrial Function in Modulating Human Granulosa Cell Steroidogenesis and Female Fertility
Urs, D. B. S., Wu, W.-H., Komrsková, K., Manaskova-Postlerova, P., Lin, Y., Tzeng, C., & Kao, S. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21103592 · Cited by 194
MECHANISMS IN ENDOCRINOLOGY: Rare defects in adrenal steroidogenesis
Miller, W. L. (2018) · European Journal of Endocrinology · doi:10.1530/eje-18-0279 · Cited by 138
Self-citation
Considering Sex as a Biological Variable in Basic and Clinical Studies: An Endocrine Society Scientific Statement
Bhargava, A., Arnold, A. P., Bangasser, D. A., Denton, K. M., Gupta, A., Hilliard Krause, L. M., Mayer, E. A., McCarthy, M., Miller, W. L., Raznahan, A., et al. (2021) · Endocrine Reviews · doi:10.1210/endrev/bnaa034 · Cited by 130
Self-citation
Oxidative stress and mitochondrial dysfunction of granulosa cells in polycystic ovarian syndrome
Gao, Y., Zou, Y., Wu, G., & Zheng, L. (2023) · Frontiers in Medicine · doi:10.3389/fmed.2023.1193749 · Cited by 128
Current knowledge on the acute regulation of steroidogenesis
Selvaraj, V., Stocco, D. M., & Clark, B. J. (2018) · Biology of Reproduction · doi:10.1093/biolre/ioy102 · Cited by 118
Biological Effects of CYP11A1-Derived Vitamin D and Lumisterol Metabolites in the Skin
Slominski, A. T., Kim, T., Janjetović, Z., Slominski, R. M., Li, W., Jetten, A. M., Indra, A. K., Mason, R. S., & Tuckey, R. C. (2024) · Journal of Investigative Dermatology · doi:10.1016/j.jid.2024.04.022 · Cited by 110
Genetics of Congenital Adrenal Hyperplasia
Hannah-Shmouni, F., Chen, W., & Merke, D. P. (2017) · Endocrinology and Metabolism Clinics of North America · doi:10.1016/j.ecl.2017.01.008 · Cited by 97
Primary adrenal insufficiency: New genetic causes and their long‐term consequences
Buonocore, F., & Achermann, J. C. (2019) · Clinical Endocrinology · doi:10.1111/cen.14109 · Cited by 92
A brief history of the search for the protein(s) involved in the acute regulation of steroidogenesis
Stocco, D. M., Zhao, A. H., Tu, L. N., Morohaku, K., & Selvaraj, V. (2016) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2016.07.036 · Cited by 85
Deficiency of the sphingosine-1-phosphate lyase SGPL1 is associated with congenital nephrotic syndrome and congenital adrenal calcifications
Janecke, A., Xu, R., Steichen‐Gersdorf, E., Waldegger, S., Entenmann, A., Giner, T., Krainer, I. M., Huber, L. A., Hess, M. W., Frishberg, Y., et al. (2017) · Human Mutation · doi:10.1002/humu.23192 · Cited by 83
Monogenic disorders of adrenal steroidogenesis
Baranowski, E. S., Arlt, W., & Idkowiak, J. (2018) · Hormone Research in Paediatrics · doi:10.1159/000488034 · Cited by 79
Perturbations in Lineage Specification of Granulosa and Theca Cells May Alter Corpus Luteum Formation and Function
Abedal-Majed, M. A., Romereim, S. M., Davis, J. S., & Cupp, A. S. (2019) · Frontiers in Endocrinology · doi:10.3389/fendo.2019.00832 · Cited by 79
Function of CYP11A1 in the mitochondria
Chien, Y., Rosal, K. G., & Chung, B. (2016) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2016.10.030 · Cited by 75
Mitochondrial GSH replenishment as a potential therapeutic approach for Niemann Pick type C disease
Torres, S., Matías, N., Baulies, A., Núñez, S., Alarcón‐Vila, C., Martínez-Gili, L., Nuño‐Lámbarri, N., Fernandez, A., Caballería, J., Levade, T., et al. (2016) · Redox Biology · doi:10.1016/j.redox.2016.11.010 · Cited by 71
The Steroidogenic Acute Regulatory Protein (StAR) Is Regulated by the H19/let-7 Axis
Men, Y., Fan, Y., Shen, Y., Lu, L., & Kallen, A. N. (2016) · Endocrinology · doi:10.1210/en.2016-1340 · Cited by 70
Biosynthesis and signalling functions of central and peripheral nervous system neurosteroids in health and disease
Lloyd‐Evans, E., & Waller‐Evans, H. (2020) · Essays in Biochemistry · doi:10.1042/ebc20200043 · Cited by 72
Role of Granulosa Cells in the Aging Ovarian Landscape: A Focus on Mitochondrial and Metabolic Function
Alberico, H., & Woods, D. C. (2022) · Frontiers in Physiology · doi:10.3389/fphys.2021.800739 · Cited by 69
Thoughts on interactions between PGRMC1 and diverse attested and potential hydrophobic ligands
Cahill, M. A., & Medlock, A. E. (2017) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2016.12.020 · Cited by 60
Luteinizing Hormone Regulation of Inter-Organelle Communication and Fate of the Corpus Luteum
Przygrodzka, E., Plewes, M. R., & Davis, J. S. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22189972 · Cited by 71
Disorders of Sex Development of Adrenal Origin
Finkielstain, G. P., Vieites, A., Bergadá, I., & Rey, R. A. (2021) · Frontiers in Endocrinology · doi:10.3389/fendo.2021.770782 · Cited by 60
Mitochondrial functions and rare diseases
Dard, L., Blanchard, W., Hubert, C., Lacombe, D., & Rossignol, R. (2020) · Molecular Aspects of Medicine · doi:10.1016/j.mam.2019.100842 · Cited by 65
Adrenal cortex development and related disorders leading to adrenal insufficiency
Pignatti, E., & Flück, C. E. (2021) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2021.111206 · Cited by 67
Male Hypogonadism and Disorders of Sex Development
Grinspon, R. P., Bergadá, I., & Rey, R. A. (2020) · Frontiers in Endocrinology · doi:10.3389/fendo.2020.00211 · Cited by 59
Steroidogenic Acute Regulatory Protein: Structure, Functioning, and Regulation
Tugaeva, K. V., & Sluchanko, N. N. (2019) · Biochemistry (Moscow) · doi:10.1134/s0006297919140141 · Cited by 59
Overview of the Molecular Steps in Steroidogenesis of the GABAergic Neurosteroids Allopregnanolone and Pregnanolone
Liang, J. J., & Rasmusson, A. M. (2018) · Chronic Stress · doi:10.1177/2470547018818555 · Cited by 57
The Adrenal Cortex and its Disorders
Miller, W. L. (2009) · Wiley eBooks · doi:10.1002/9781444316728.ch13 · Cited by 51
Self-citation
Mitochondria-Mediated Cardiovascular Benefits of Sodium-Glucose Co-Transporter 2 Inhibitors
Dabravolski, S. A., Zhuravlev, A. D., Kartuesov, A. G., Borisov, E. E., Sukhorukov, V. N., & Orekhov, A. N. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23105371 · Cited by 50
Current Insights Into Adrenal Insufficiency in the Newborn and Young Infant
Buonocore, F., McGlacken-Byrne, S. M., del Valle, I., & Achermann, J. C. (2020) · Frontiers in Pediatrics · doi:10.3389/fped.2020.619041 · Cited by 53
Thirty-Eight-Year Follow-Up of Two Sibling Lipoid Congenital Adrenal Hyperplasia Patients Due to Homozygous Steroidogenic Acute Regulatory (STARD1) Protein Mutation. Molecular Structure and Modeling of the STARD1 L275P Mutation
Khoury, K., Barbar, É., Ainmelk, Y., Ouellet, A., Lavigne, P., & Lehoux, J. (2016) · Frontiers in Neuroscience · doi:10.3389/fnins.2016.00527 · Cited by 40
Trafficking of cholesterol from lipid droplets to mitochondria in bovine luteal cells: Acute control of progesterone synthesis
Plewes, M. R., Krause, C. M., Talbott, H. A., Przygrodzka, E., Wood, J. R., Cupp, A. S., & Davis, J. S. (2020) · The FASEB Journal · doi:10.1096/fj.202000671r · Cited by 36
Androgen Excess Induced Mitochondrial Abnormality in Ovarian Granulosa Cells in a Rat Model of Polycystic Ovary Syndrome
Song, L., Yu, J., Zhang, D., Li, X., Chen, L., Cai, Z., & Yu, C. (2022) · Frontiers in Endocrinology · doi:10.3389/fendo.2022.789008 · Cited by 38
Intracellular cholesterol transport proteins: roles in health and disease
Soffientini, U., & Graham, A. (2016) · Clinical Science · doi:10.1042/cs20160339 · Cited by 37
Luteinizing hormone regulates the phosphorylation and localization of the mitochondrial effector dynamin‐related protein‐1 (DRP1) and steroidogenesis in the bovine corpus luteum
Plewes, M. R., Hou, X., Talbott, H. A., Zhang, P., Wood, J. R., Cupp, A. S., & Davis, J. S. (2020) · The FASEB Journal · doi:10.1096/fj.201902958r · Cited by 37
Role of the mtDNA Mutations and Mitophagy in Inflammaging
Dabravolski, S. A., Nikiforov, N. G., Zhuravlev, A. D., Orekhov, N. A., Grechko, A. V., & Orekhov, A. N. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23031323 · Cited by 39
Formation and characterization of lipid droplets of the bovine corpus luteum
Talbott, H. A., Plewes, M. R., Krause, C. M., Hou, X., Zhang, P., Rizzo, W. B., Wood, J. R., Cupp, A. S., & Davis, J. S. (2020) · Scientific Reports · doi:10.1038/s41598-020-68091-2 · Cited by 33
Single-Cell RNA-Seq Identifies Pathways and Genes Contributing to the Hyperandrogenemia Associated with Polycystic Ovary Syndrome
Harris, R. A., McAllister, J. M., & Strauss, J. F. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms241310611 · Cited by 33
Adrenocortical hyperplasia: a review of clinical presentation and imaging
Morani, A. C., Jensen, C. T., Habra, M. A., Agrons, M. M., Menias, C. O., Wagner‐Bartak, N. A., Shaaban, A. M., Roman-Colon, A. M., & Elsayes, K. M. (2019) · Abdominal Radiology · doi:10.1007/s00261-019-02048-6 · Cited by 32
STARD1 Functions in Mitochondrial Cholesterol Metabolism and Nascent HDL Formation. Gene Expression and Molecular mRNA Imaging Show Novel Splicing and a 1:1 Mitochondrial Association
Larsen, M. C., Lee, J., Jorgensen, J. S., & Jefcoate, C. R. E. (2020) · Frontiers in Endocrinology · doi:10.3389/fendo.2020.559674 · Cited by 31
Acid ceramidase improves mitochondrial function and oxidative stress in Niemann-Pick type C disease by repressing STARD1 expression and mitochondrial cholesterol accumulation
Torres, S., Solsona‐Vilarrasa, E., Núñez, S., Matías, N., Insausti-Urkia, N., Castro, F., Casasampere, M., Fabriàs, G., Casas, J., Enrich, C., et al. (2021) · Redox Biology · doi:10.1016/j.redox.2021.102052 · Cited by 32
Pediatric Adrenal Insufficiency: Challenges and Solutions
Nisticò, D., Bossini, B., Benvenuto, S., Pellegrin, M. C., & Tornese, G. (2022) · Therapeutics and Clinical Risk Management · doi:10.2147/tcrm.s294065 · Cited by 35
Update on adrenal steroid hormone biosynthesis and clinical implications
Bacila, I. A., Elder, C. J., & Krone, N. P. (2019) · Archives of Disease in Childhood · doi:10.1136/archdischild-2017-313873 · Cited by 30
History of Adrenal Research: From Ancient Anatomy to Contemporary Molecular Biology
Miller, W. L., & White, P. C. (2022) · Endocrine Reviews · doi:10.1210/endrev/bnac019 · Cited by 32
Self-citation
Quantitative proteomic analysis of the liver reveals antidepressant potential protein targets of Sinisan in a mouse CUMS model of depression
Wang, B., Lu, S., Zhang, C., Zhu, L., Li, Y., Bai, M., & Xu, E. (2020) · Biomedicine & Pharmacotherapy · doi:10.1016/j.biopha.2020.110565 · Cited by 29
Aberrant Splicing Is the Pathogenicity Mechanism of the p.Glu314Lys Variant in CYP11A1 Gene
Goursaud, C., Mallet, D., Janin, A., Menassa, R., Tardy-Guidollet, V., Russo, G., Lienhardt-Roussie, A., Lecointre, C., Plotton, I., Morel, Y., et al. (2018) · Frontiers in Endocrinology · doi:10.3389/fendo.2018.00491 · Cited by 29
Mutation Spectrum of STAR and the Founder Effect of p.Q258* in Korean Patients with Congenital Lipoid Adrenal Hyperplasia
Kang, E., Kim, Y.-M., Kim, G.-H., Lee, B. H., Yoo, H., & Choi, J. (2017) · Molecular Medicine · doi:10.2119/molmed.2017.00023 · Cited by 30
Steroids Bearing Heteroatom as Potential Drugs for Medicine
Dembitsky, V. M. (2023) · Biomedicines · doi:10.3390/biomedicines11102698 · Cited by 28
An altered lipid metabolism characterizes Charcot-Marie-Tooth type 2B peripheral neuropathy
Giudetti, A. M., Guerra, F., Longo, S., Beli, R., Romanò, R., Manganelli, F., Nolano, M., Mangini, V., Santoro, L., & Bucci, C. (2020) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2020.158805 · Cited by 29
Congenital Adrenal Hyperplasias Presenting in the Newborn and Young Infant
Balsamo, A., Baronio, F., Ortolano, R., Menabò, S., Baldazzi, L., Natale, V. D., Vissani, S., & Cassio, A. (2020) · Frontiers in Pediatrics · doi:10.3389/fped.2020.593315 · Cited by 27
MEHP induces alteration of mitochondrial function and inhibition of steroid biosynthesis in MA-10 mouse tumor Leydig cells
Traore, K., More, P., Adla, A., Dogbey, G. Y., Papadopoulos, V., & Zirkin, B. R. (2021) · Toxicology · doi:10.1016/j.tox.2021.152985 · Cited by 25
Steroidogenic electron-transfer factors and their diseases
Miller, W. L. (2021) · Annals of Pediatric Endocrinology & Metabolism · doi:10.6065/apem.2142154.077 · Cited by 24
Self-citation
Prenatal amoxicillin exposure induces developmental toxicity in fetal mice and its characteristics
Dai, Y., Peng, Y., Hu, W., Liu, Y., & Wang, H. (2023) · Journal of Environmental Sciences · doi:10.1016/j.jes.2023.02.021 · Cited by 23
Development of a prioritization method for chemical-mediated effects on steroidogenesis using an integrated statistical analysis of high-throughput H295R data
Haggard, D. E., Setzer, R. W., Judson, R., & Friedman, K. P. (2019) · Regulatory Toxicology and Pharmacology · doi:10.1016/j.yrtph.2019.104510 · Cited by 23
Rare forms of congenital adrenal hyperplasia
Tosun, B. G., & Güran, T. (2023) · Clinical Endocrinology · doi:10.1111/cen.15009 · Cited by 26
A mixture of persistent organic pollutants detected in human follicular fluid increases progesterone secretion and mitochondrial activity in human granulosa HGrC1 cells
Krawczyk, K., Marynowicz, W., Gogola-Mruk, J., Jakubowska, K., Tworzydlo, W., Opydo‐Chanek, M., & Ptak, A. (2021) · Reproductive Toxicology · doi:10.1016/j.reprotox.2021.07.009 · Cited by 24
Comprehensive Metabolomics Analysis of Xueshuan Xinmaining Tablet in Blood Stasis Model Rats Using UPLC-Q/TOF-MS
Tan, J. H., Wang, C., Zhu, H., Zhou, B., Xiong, L., Wang, F., Li, P., & Liu, J. (2018) · Molecules · doi:10.3390/molecules23071650 · Cited by 21
Dynamic urinary metabolomics analysis based on UHPLC-Q-TOF/MS to investigate the potential biomarkers of blood stasis syndrome and the effects of Danggui Sini decoction
Huang, H., Wu, J., Lu, R., Liu, X., Chin, B., Zhu, H., Yin, C., Cheng, B., Wu, Z., Chen, X., et al. (2019) · Journal of Pharmaceutical and Biomedical Analysis · doi:10.1016/j.jpba.2019.112986 · Cited by 20
Royal jelly reduces depression-like behavior through possible effects on adrenal steroidogenesis in a murine model of unpredictable chronic mild stress
Iegaki, N., Narita, Y., Hattori, N., Hirata, Y., & Ichihara, K. (2019) · Bioscience Biotechnology and Biochemistry · doi:10.1080/09168451.2019.1691496 · Cited by 21
Epoxyeicosatrienoic acids: Emerging therapeutic agents for central post-stroke pain
Wan, L., Li, Z., Liu, T., Chen, X., Xu, Q., Yao, W., Zhang, C., & Zhang, Y. (2020) · Pharmacological Research · doi:10.1016/j.phrs.2020.104923 · Cited by 21
Estrogens and development of the mouse and human external genitalia
Baskin, L. S., Sinclair, A. W., Derpinghaus, A., Cao, M., Li, Y., Overland, M. R., Aksel, S., & Cunha, G. R. (2020) · Differentiation · doi:10.1016/j.diff.2020.09.004 · Cited by 21
Pregnancy and Prenatal Management of Congenital Adrenal Hyperplasia
Cera, G., Locantore, P., Novizio, R., Maggio, E., Ramunno, V., Corsello, A., Policola, C., Concolino, P., Paragliola, R. M., & Pontecorvi, A. (2022) · Journal of Clinical Medicine · doi:10.3390/jcm11206156 · Cited by 23
The ROS/SIRT1/STAR axis as a target for melatonin ameliorating atrazine-induced mitochondrial dysfunction and steroid disorders in granulosa cells
Li, X., Yi, B., Wang, Z., Guo, K., Saleem, M. A. U., Ma, X., Li, X.-N., & Li, J. (2023) · Ecotoxicology and Environmental Safety · doi:10.1016/j.ecoenv.2023.115780 · Cited by 21
Electron Transport Chain Complex II Regulates Steroid Metabolism
Bose, H. S., Marshall, B., Debnath, D. K., Perry, E. W., & Whittal, R. M. (2020) · iScience · doi:10.1016/j.isci.2020.101295 · Cited by 21
Puberty in the Female and Its Disorders
Rosenfield, R. L., Cooke, D. W., & Radovick, S. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-62520-3.00016-6 · Cited by 23
Paternal cadmium exposure affects estradiol synthesis by impairing intracellular cholesterol homeostasis and mitochondrial function in offspring female mice
Zhang, Y., Zhou, J., Zeng, L., Xiong, Y., Wang, X., Xiang, W., & Su, P. (2023) · Ecotoxicology and Environmental Safety · doi:10.1016/j.ecoenv.2023.115280 · Cited by 21
Pig StAR: mRNA expression and alternative splicing in testis and Leydig cells, and association analyses with testicular morphology traits
Zhang, Y., Cui, Y., Zhang, X., Wang, Y., Gao, J., Yu, T. Y., Lv, X., & Pan, C. (2018) · Theriogenology · doi:10.1016/j.theriogenology.2018.05.031 · Cited by 19
Mutational analysis of rare subtypes of congenital adrenal hyperplasia in a highly inbred population
Alswailem, M. M., Alzahrani, O., Alhomaidah, D. S., Alasmari, R., Qasem, E., Murugan, A. K., Alsagheir, A., Brema, I. A., Abbas, B. B., Almehthel, M. S., et al. (2017) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2017.08.022 · Cited by 19
Pig Hsd17b3: Alternative splice variants expression, insertion/deletion (indel) in promoter region and their associations with male reproductive traits
Chen, M., Yang, W., Liu, N., Zhang, X., Dong, W., Lan, X., & Pan, C. (2019) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2019.105483 · Cited by 19
CYP19A1 May Influence Lambing Traits in Goats by Regulating the Biological Function of Granulosa Cells
Zhang, Y., Chen, X., Zhou, Z., Tian, X., Yang, P., & Fu, K. (2022) · Animals · doi:10.3390/ani12151911 · Cited by 18
Towards Improved Steroid Hormone Analysis: The Shift from Immunoassays to Mass Spectrometry
Deng, B., Bi, M., Zheng, Y., Hussain, D., Yang, S., & Chen, D. (2024) · Journal of Analysis and Testing · doi:10.1007/s41664-024-00326-x · Cited by 21
Steroid Hormones and Other Lipid Molecules Involved in Human Reproduction
Strauss, J. F., & FitzGerald, G. A. (2019) · Elsevier eBooks · doi:10.1016/b978-0-323-47912-7.00004-4 · Cited by 21
Glucocorticoid deficiency causes transcriptional and post-transcriptional reprogramming of glutamine metabolism
Weger, M., Weger, B. D., Görling, B., Poschet, G., Yıldız, M., Hell, R., Luy, B., Akçay, T., Güran, T., Dickmeis, T., et al. (2018) · EBioMedicine · doi:10.1016/j.ebiom.2018.09.024 · Cited by 20
An overview of inborn errors of metabolism manifesting with primary adrenal insufficiency
Hannah‐Shmouni, F., & Stratakis, C. A. (2018) · Reviews in Endocrine and Metabolic Disorders · doi:10.1007/s11154-018-9447-2 · Cited by 17
The Steroidogenic Acute Regulatory (STAR) Gene Anatomy, Expression, and Roles
Lim, J., & Cheon, Y. (2025) · Development & Reproduction · doi:10.12717/dr.2025.29.3.63 · Cited by 17
Angiotensin-(1-7), Angiotensin-Converting Enzyme 2 and Mas Receptor in Rat Polycystic Ovaries
Pereira, V. M., dos Reis, F. M., Casalechi, M., & dos Reis, A. M. (2020) · Protein and Peptide Letters · doi:10.2174/0929866527666200826104410 · Cited by 16
Clinical and molecular characterization of thirty Chinese patients with congenital lipoid adrenal hyperplasia
Zhang, T., Ma, X., Wang, J., Jia, C., Wang, W., Dong, Z., Ye, L., Sun, S., Hu, R., Ning, G., et al. (2020) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/j.jsbmb.2020.105788 · Cited by 18
Gender differences in susceptibility to schizophrenia: Potential implication of neurosteroids
Huang, Y., Hung, C., Lin, P., Lee, Y., Wu, C., Hsu, S., Chen, C.-C., Chong, M., Lin, C., & Wang, L. (2017) · Psychoneuroendocrinology · doi:10.1016/j.psyneuen.2017.06.017 · Cited by 17
A novel splice site variant in CYP 11A1 in trans with the p.E314K variant in a male patient with congenital adrenal insufficiency
Lara-Velazquez, M. A., Perdomo‐Pantoja, A., Blackburn, P. R., Gass, J. M., Caulfield, T. R., & Atwal, P. S. (2017) · Molecular Genetics & Genomic Medicine · doi:10.1002/mgg3.322 · Cited by 17
Steroid Hormones: Synthesis, Secretion, and Transport
Acconcia, F., & Marino, M. (2016) · Endocrinology · doi:10.1007/978-3-319-27318-1_4-1 · Cited by 18
Hyperandrogenic environment regulates the function of ovarian granulosa cells by modulating macrophage polarization in PCOS
Chen, X., Hong, L., Diao, L., Yin, T., & Liu, S. (2024) · American Journal of Reproductive Immunology · doi:10.1111/aji.13854 · Cited by 17
A Novel Mitochondrial Complex of Aldosterone Synthase, Steroidogenic Acute Regulatory Protein, and Tom22 Synthesizes Aldosterone in the Rat Heart
Bose, H. S., Whittal, R. M., Marshall, B., Rajapaksha, M., Wang, N., Bose, M., Perry, E. W., Zhao, Z., & Miller, W. L. (2021) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.120.000365 · Cited by 16
Self-citation
Histopathological and transcriptomic analyses reveal the reproductive endocrine-disrupting effects of decabromodiphenyl ethane (DBDPE) in mussel Mytilus galloprovincialis
Wang, S., Wang, Z., Wang, X., Qu, J., Li, F., Ji, C., & Wu, H. (2022) · The Science of The Total Environment · doi:10.1016/j.scitotenv.2022.160724 · Cited by 15
The Current and Emerging Role of Statins in the Treatment of PCOS: The Evidence to Date
Kolnikaj, T. S., Herman, R., Janež, A., & Jensterle, M. (2024) · Medicina · doi:10.3390/medicina60020244 · Cited by 18
A rare cause of primary adrenal insufficiency due to a homozygous Arg188Cys mutation in the STAR gene
Burget, L., Audı́, L., Fernández‐Cancio, M., Gräni, R., Henzen, C., & Flück, C. E. (2018) · Endocrinology Diabetes and Metabolism Case Reports · doi:10.1530/edm-18-0003 · Cited by 16
Metabolic Alteration Analysis of Steroid Hormones in Niemann–Pick Disease Type C Model Cell Using Liquid Chromatography/Tandem Mass Spectrometry
Abe, A., Maekawa, M., Sato, T., Sato, Y., Kumondai, M., Takahashi, H., Kikuchi, M., Higaki, K., Ogura, J., & Mano, N. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms23084459 · Cited by 14
Luteal Lipid Droplets: A Novel Platform for Steroid Synthesis
Plewes, M. R., Talbott, H. A., Saviola, A. J., Woods, N. T., Schott, M. B., & Davis, J. S. (2023) · Endocrinology · doi:10.1210/endocr/bqad124 · Cited by 14
CORO1A regulates lipoprotein uptake in Leydig cells exposed to cadmium
Wang, Y., Li, T., Li, H., Liang, Y., Mai, W., Liu, C., Chen, H., Huang, Y., & Zhang, Q. (2022) · Ecotoxicology and Environmental Safety · doi:10.1016/j.ecoenv.2022.113255 · Cited by 14
The Adrenal Cortex and Its Disorders
Miller, W. L., Flück, C. E., Breault, D. T., & Feldman, B. J. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-62520-3.00014-2 · Cited by 14
Self-citation
Protective function of StAR in amyloid-β accumulated hippocampal neurotoxicity and neurosteroidogenesis: Mechanistic insights into Alzheimer's disease
Manna, P. R., Kshirsagar, S., Pradeepkiran, J. A., Rawat, P., Kumar, S., Reddy, A. P., & Reddy, P. H. (2023) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2023.166738 · Cited by 14
Lipoid congenital adrenal hyperplasia by steroidogenic acute regulatory protein (STAR) gene mutation in an Italian infant: an uncommon cause of adrenal insufficiency
Bizzarri, C., Pisaneschi, E., Mucciolo, M., Pedicelli, S., Galeazzi, D., Novelli, A., & Cappa, M. (2017) · The Italian Journal of Pediatrics/Italian journal of pediatrics · doi:10.1186/s13052-017-0371-y · Cited by 16
Thirty years of StAR gazing. Expanding the universe of the steroidogenic acute regulatory protein
Miller, W. L. (2025) · Journal of Endocrinology · doi:10.1530/joe-24-0310 · Cited by 16
Self-citation
Clinical characteristics of a male child with non-classic lipoid congenital adrenal hyperplasia and literature review
Lü, W., Zhang, T., Zhang, L., Wang, X., Lv, S., Wang, J., Ye, L., Xiao, Y., Dong, Z., Wang, W., et al. (2022) · Frontiers in Endocrinology · doi:10.3389/fendo.2022.947762 · Cited by 14
Which is the current knowledge on man-made endocrine- disrupting chemicals in follicular fluid? An overview of effects on ovarian function and reproductive health
Shulhai, A.-M., Bianco, V., Donini, V., Esposito, S., & Street, M. E. (2024) · Frontiers in Endocrinology · doi:10.3389/fendo.2024.1435121 · Cited by 13
Induction of steroidogenic cells from adult stem cells and pluripotent stem cells [Review]
Yazawa, T., Imamichi, Y., Miyamoto, K., Khan, M. R. I., Uwada, J., Umezawa, A., & Taniguchi, T. (2016) · Endocrine Journal · doi:10.1507/endocrj.ej16-0373 · Cited by 13
Effect of the NBD-group position on interaction of fluorescently-labeled cholesterol analogues with human steroidogenic acute regulatory protein STARD1
Tugaeva, K. V., Faletrov, Y. V., Allakhverdiev, E. S., Shkumatov, V. M., Maksimov, E. G., & Sluchanko, N. N. (2018) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2018.02.014 · Cited by 14
Disorders of the adrenal cortex: Genetic and molecular aspects
Pitsava, G., Maria, A. G., & Faucz, F. R. (2022) · Frontiers in Endocrinology · doi:10.3389/fendo.2022.931389 · Cited by 13
Adrenal Insufficiency, Sex Reversal, and Angelman Syndrome due to Uniparental Disomy Unmasking a Mutation in CYP11A1
Kim, A., Fujimoto, M., Hwa, V., Backeljauw, P. F., & Dauber, A. N. (2018) · Hormone Research in Paediatrics · doi:10.1159/000487638 · Cited by 13

Authors: Miller, W. L.
Affiliations: University of California, San Francisco (US)
Corresponding author: Miller, Walter L.
ORCID: Miller, Walter L.
Venue: The Journal of Steroid Biochemistry and Molecular Biology
Publisher: Elsevier BV
Published: 2017-01
Volume: 165
Issue: Pt A
Pages: 18-37
ISSN: 0960-0760 (ISSN-L)
DOI: 10.1016/j.jsbmb.2016.03.009
OpenAlex: W2298595356
Semantic Scholar: fe8733103b7e517d495e8f1bac7db018713287f1
Cited by: 205
References: 272
Citations by year: 2026: 6 · 2025: 23 · 2024: 22 · 2023: 34 · 2022: 23 · 2021: 22 · 2020: 21 · 2019: 15 · 2018: 18 · 2017: 11
FWCI: 2.53
Type: journal article
Language: en
Open access: closed
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: The Journal of Steroid Biochemistry and Molecular Biology (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Sexual Differentiation and Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Keywords: steroidogenesis, LDL receptor, adrenoleukodystrophy, Wolman disease, cholesterol ester storage disease, Niemann-Pick disease type C, adrenal insufficiency, steroidogenic acute regulatory protein, congenital lipoid adrenal hyperplasia, cholesterol side-chain cleavage enzyme, P450scc, CYP11A1, adrenal hyperplasia, adrenal imaging, glucocorticoid deficiency, mineralocorticoid deficiency, sex steroid synthesis, disorders of sex development
MeSH terms: Steroidogenic Acute Regulatory Protein, Adrenal Glands, Adrenal Hyperplasia, Congenital, Animals, Cholesterol, Cholesterol Side-Chain Cleavage Enzyme, Computer Simulation, Cytochrome P-450 Enzyme System, Female, Glucocorticoids, Humans, Male, Mitochondria, Mutation, Phosphoproteins, Steroids, Mutagenesis, Sequence Analysis, DNA, Disorder of Sex Development, 46,XY

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Disorders in the initial steps of steroid hormone synthesis

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Citations

Cites

Cited by

Related

Metadata

Record

About these checks