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Details on Person UniProt:Q9NSC2 SALL1

Class:IdReferenceGeneProduct:64120
_chainChangeLogchain:1-1324 added on Fri February 6 2015
_displayNameUniProt:Q9NSC2 SALL1
_timestamp2025-02-21 20:14:18
chainchain:1-1324
checksum41AFA91ADEBEEF8C
commentFUNCTION Transcriptional repressor involved in organogenesis. Plays an essential role in ureteric bud invasion during kidney development.SUBUNIT May associate with NuRD histone deacetylase complex (HDAC) (By similarity). Interacts with components of HDAC complex including HDAC1, HDAC2, RBBP4, RBPP7, MTA1 and MTA2 (By similarity). Interacts with CCNQ (PubMed:18297069). Interacts with NSD2 (via PHD-type zinc fingers 1, 2 and 3) (By similarity).INTERACTION Highest levels in kidney. Lower levels in adult brain (enriched in corpus callosum, lower expression in substantia nigra) and liver.DEVELOPMENTAL STAGE In fetal brain exclusively in neurons of the subependymal region of hypothalamus lateral to the third ventricle.DISEASE The disease is caused by variants affecting the gene represented in this entry. The pathogenic mechanism includes abnormal interactions of mutant SALL1 protein with proteins that control cilium formation and function, resulting in ciliary defects (PubMed:32553112). Some individuals with SALL1 mutations manifest a phenotype overlapping with TBS1 and bronchio-oto-renal syndrome (PubMed:10928856). Clinical features include dysplastic ears, hypoplastic kidneys with impaired renal function, gastroesophageal reflux, hypermetropia, hypospadias, and mild developmental delay (PubMed:10928856). Affected individuals lack the characteristic anal or hand malformations of TBS1 (PubMed:10928856).SIMILARITY Belongs to the sal C2H2-type zinc-finger protein family.
descriptionrecommendedName: Sal-like protein 1 alternativeName: Spalt-like transcription factor 1 alternativeName: Zinc finger protein 794 alternativeName: Zinc finger protein SALL1 alternativeName: Zinc finger protein Spalt-1 shortName: HSal1 shortName: Sal-1
geneNameSALL1
SAL1
ZNF794
identifierQ9NSC2
isSequenceChangedFALSE
keywordAlternative splicing
Deafness
DNA-binding
Isopeptide bond
Metal-binding
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
Repressor
Transcription
Transcription regulation
Ubl conjugation
Zinc
Zinc-finger
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameSALL1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:2888905] ENSEMBL:ENSG00000103449 SALL1 [Homo sapiens]
secondaryIdentifierSALL1_HUMAN
Q99881
Q9NSC3
Q9P1R0
sequenceLength1324
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:8972556] UniProt:Q9NSC2-1 SALL1 [Homo sapiens]
[ReferenceIsoform:8972557] UniProt:Q9NSC2-2 SALL1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:452500] SALL1 [nucleoplasm] [Homo sapiens]
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