Cited by 181Sphingolipid Metabolism and SignalingLysosomal Storage Disorders ResearchLipid metabolism and biosynthesis

TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?

Winter, Eitan, Ponting, Chris P·2002·Trends in Biochemical Sciences

Abstract

A family of membrane-associated proteins related to yeast Lag1p and mammalian TRAM has been identified. The family includes the protein product of CLN8, a gene mutated in progressive epilepsy with mental retardation. Mouse CLN8 is also mutated in the mnd/mnd mouse, a model for neuronal ceroid lipofuscinoses. The identification of these homologues has potential implications for our understanding of ceramide synthesis, lipid regulation and protein translocation in the endoplasmic reticulum.

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Crossref · 10.1016/s0968-0004(02)02154-0

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  • There is a cysteine in an extension following the predicted ADAM33 prodomain barrel; taken together, these data are consistent with experimental data suggesting that the ADAM prodomain inhibits metalloprotease activity using a cysteine switch.

    “There is a cysteine in an extension following the predicted prodomain barrel; taken together, these data are consistent with experimental data suggesting that the ADAM prodomain inhibits metalloprotease activity using a cysteine switch (26).”

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Authors
Winter, E., & Ponting, C. P.
Affiliations
University of Oxford (GB)
Corresponding author
Ponting, Chris P
Venue
Trends in Biochemical Sciences
Publisher
Elsevier BV
Published
2002-08
Volume
27
Issue
8
Pages
381-383
ISSN
0968-0004 (ISSN-L)
OpenAlex
W2016874659
Cited by
181
References
22
Citations by year
2026: 2 · 2025: 3 · 2024: 8 · 2023: 4 · 2022: 4 · 2021: 9 · 2020: 9 · 2019: 8 · 2018: 8 · 2017: 4 · 2016: 11 · 2015: 4 · 2014: 5 · 2013: 10 · 2012: 16
FWCI
0.55
Type
journal article
Language
en
Open access
closed
Locations
  • Trends in Biochemical Sciences (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sphingolipid Metabolism and Signaling
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
Keywords
tram, CLN8, membrane-associated proteins, ceramide synthesis, lipid regulation, neuronal ceroid lipofuscinosis, protein families
MeSH terms
Amino Acid Sequence, Animals, Fungal Proteins, Humans, Membrane Glycoproteins, Membrane Proteins, Molecular Sequence Data, Mutation, Sequence Homology, Amino Acid, Protein Structure, Tertiary, Membrane Transport Proteins, Saccharomyces cerevisiae Proteins, Lipid Metabolism, Mice

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