TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?
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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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).”
From Accurate prediction of protein structures and interactions using a 3-track network (2021) · cites this paper as [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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