title: 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.
authors.affiliation: MRC Functional Genetics Unit, Department of Human Anatomy and Genetics, University of Oxford, South Parks Road, Oxford, UK OX1 3QX.
authors.forename: Eitan
authors.initials: E
authors.lastname: Winter
authors.forename: Chris P
authors.initials: CP
authors.lastname: Ponting
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D005656:Fungal Proteins / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D050356:Lipid Metabolism; D008562:Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D026901:Membrane Transport Proteins; D051379:Mice; D008969:Molecular Sequence Data; D009154:Mutation; D017434:Protein Structure, Tertiary; D029701:Saccharomyces cerevisiae Proteins; D017386:Sequence Homology, Amino Acid
chemical_list: C121767:CLN8 protein, human; C498493:Cln8 protein, mouse; D005656:Fungal Proteins; C087436:LAG1 protein, S cerevisiae; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D026901:Membrane Transport Proteins; D029701:Saccharomyces cerevisiae Proteins; C486174:TRAM1 protein, human; C074282:translocating chain-associating membrane protein (TRAM)