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Details on Person UniProt:P32004-1 L1CAM

Class:IdReferenceIsoform:403662
_chainChangeLogsignal peptide:1-19 added on Sat February 7 2015;chain:20-1257 added on Sat February 7 2015
_displayNameUniProt:P32004-1 L1CAM
_timestamp2026-02-20 22:45:15
chainsignal peptide:1-19
chain:20-1257
checksum5EDD764DA86C0E63
commentFUNCTION Neural cell adhesion molecule involved in the dynamics of cell adhesion and in the generation of transmembrane signals at tyrosine kinase receptors. During brain development, critical in multiple processes, including neuronal migration, axonal growth and fasciculation, and synaptogenesis. In the mature brain, plays a role in the dynamics of neuronal structure and function, including synaptic plasticity.SUBUNIT Interacts with SHTN1; the interaction occurs in axonal growth cones (By similarity). Interacts with isoform 2 of BSG (By similarity).INTERACTION Colocalized with SHTN1 in close apposition with actin filaments in filopodia and lamellipodia of axonalne growth cones of hippocampal neurons (By similarity). In neurons, detected predominantly in axons and cell body, weak localization to dendrites (PubMed:20621658).ALTERNATIVE PRODUCTS The disease is caused by variants affecting the gene represented in this entry. L1CAM mutations have also been found in few patients affected by hydrocephalus with Hirschsprung disease, suggesting a role of this gene acting either in a direct or indirect way in the pathogenesis of Hirschsprung disease (PubMed:22344793).DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE Defects in L1CAM may contribute to Hirschsprung disease by modifying the effects of Hirschsprung disease-associated genes to cause intestinal aganglionosis.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE Defects in L1CAM are associated with a wide phenotypic spectrum which varies from severe hydrocephalus and prenatal death (HYCX) to a milder phenotype (MASA). These variations may even occur within the same family. Due to the overlap of phenotypes between HYCX and MASA, many authors use the general concept of L1 syndrome which covers both ends of the spectrum.SIMILARITY Belongs to the immunoglobulin superfamily. L1/neurofascin/NgCAM family.ONLINE INFORMATION L1CAM mutation Web Page
created[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
descriptionrecommendedName: Neural cell adhesion molecule L1 shortName: N-CAM-L1 shortName: NCAM-L1 cdAntigenNameCD171/cdAntigenName
geneNameL1CAM
CAML1
MIC5
identifierP32004
isoformParent
isSequenceChangedFALSE
keyword3D-structure
Alternative splicing
Cell adhesion
Cell membrane
Cell projection
Developmental protein
Differentiation
Direct protein sequencing
Disease variant
Disulfide bond
Glycoprotein
Hereditary spastic paraplegia
Hirschsprung disease
Immunoglobulin domain
Intellectual disability
Membrane
Neurodegeneration
Neurogenesis
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
Signal
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9841277] Weiser, Joel
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameL1CAM
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8997241] ENSEMBL:ENSG00000198910 L1CAM [Homo sapiens]
secondaryIdentifierL1CAM_HUMAN
A0AV65
A4ZYW4
B2RMU7
G3XAF4
Q8TA87
sequenceLength1257
species[Species:48887] Homo sapiens
variantIdentifierP32004-1
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