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Details on Person UniProt:Q96J84-1 KIRREL1

Class:IdReferenceIsoform:149368
_chainChangeLogsignal peptide:1-16 added on Fri February 6 2015;chain:17-757 added on Fri February 6 2015
_displayNameUniProt:Q96J84-1 KIRREL1
_timestamp2025-08-15 22:15:34
chainsignal peptide:1-16
chain:17-757
checksumB937421970447FC2
commentFUNCTION Required for proper function of the glomerular filtration barrier. It is involved in the maintenance of a stable podocyte architecture with interdigitating foot processes connected by specialized cell-cell junctions, known as the slit diaphragm (PubMed:31472902). It is a signaling protein that needs the presence of TEC kinases to fully trans-activate the transcription factor AP-1 (By similarity).SUBUNIT Interacts with TJP1/ZO-1 and with NPHS2/podocin (via the C-terminus). Interacts with NPHS1/nephrin (via the Ig-like domains); this interaction is dependent on KIRREL1 glycosylation. Homodimer (via the Ig-like domains). Interacts when tyrosine-phosphorylated with GRB2 (By similarity).INTERACTION Predominantly located at podocyte slit diaphragm.ALTERNATIVE PRODUCTS Abundantly expressed in kidney. Specifically expressed in podocytes of kidney glomeruli.PTM Phosphorylation probably regulates the interaction with NSH2. Phosphorylated at Tyr-605 and Tyr-606 by FYN, leading to GRB2 binding (By similarity).PTM N-glycosylated.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the immunoglobulin superfamily.
created[InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
descriptionrecommendedName: Kin of IRRE-like protein 1 alternativeName: Kin of irregular chiasm-like protein 1 alternativeName: Nephrin-like protein 1
geneNameKIRREL1
KIRREL
NEPH1
identifierQ96J84
isoformParent
isSequenceChangedFALSE
keywordAlternative splicing
Cell membrane
Disease variant
Disulfide bond
Glycoprotein
Immunoglobulin domain
Membrane
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
Signal
Transmembrane
Transmembrane helix
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameKIRREL1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8998951] ENSEMBL:ENSG00000183853 KIRREL1 [Homo sapiens]
secondaryIdentifierKIRR1_HUMAN
Q5W0F8
Q5XKC6
Q7Z696
Q7Z7N8
Q8TB15
Q9H9N1
Q9NVA5
sequenceLength757
species[Species:48887] Homo sapiens
variantIdentifierQ96J84-1
(referenceEntity)[EntityWithAccessionedSequence:197826] KIRREL-1 [plasma membrane] [Homo sapiens]
[EntityWithAccessionedSequence:374553] p-Y605,Y606-KIRREL [plasma membrane] [Homo sapiens]
(referenceSequence)[ModifiedResidue:374537] O4'-phospho-L-tyrosine at 605
[ModifiedResidue:374539] O4'-phospho-L-tyrosine at 606
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No pathways have been reviewed or authored by UniProt:Q96J84-1 KIRREL1 (149368)