UniProt:Q8IWL3 HSCB

chain
  • chain:1-235
  • chain:30-235
checksum 70CF499E58FFD1C2
comment
  • FUNCTION Acts as a co-chaperone in iron-sulfur cluster assembly in mitochondria (PubMed:20668094). Required for incorporation of iron-sulfur clusters into SDHB, the iron-sulfur protein subunit of succinate dehydrogenase that is involved in complex II of the mitochondrial electron transport chain (PubMed:26749241). Recruited to SDHB by interaction with SDHAF1 which first binds SDHB and then recruits the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20 (PubMed:26749241). Plays an essential role in hematopoiesis (By similarity).FUNCTION Acts as a co-chaperone in iron-sulfur cluster assembly in the cytoplasm (PubMed:29309586). Also mediates complex formation between components of the cytosolic iron-sulfur biogenesis pathway and the CIA targeting complex composed of CIAO1, DIPK1B/FAM69B and MMS19 by binding directly to the scaffold protein ISCU and to CIAO1 (PubMed:29309586). This facilitates iron-sulfur cluster insertion into a number of cytoplasmic and nuclear proteins including POLD1, ELP3, DPYD and PPAT (PubMed:29309586).PATHWAY Cofactor biosynthesis; iron-sulfur cluster biosynthesis.SUBUNIT Interacts with ISCU and HSPA9 to form an iron-sulfur transfer complex (PubMed:20668094). Interacts with SDHAF1 (via the first LYR motif); the interaction recruits the iron-sulfur transfer complex composed of HSC20, HSPA9 and ISCU and mediates the incorporation of iron-sulfur clusters into SDHB which also interacts with HSC20 (PubMed:26749241). Interacts with the cytoplasmic form of ISCU and with CIA complex member CIAO1 (via LYR motif) (PubMed:29309586).SUBUNIT Homodimer (PubMed:29309586). Interacts with ISCU (cytoplasmic form); this interaction stabilizes the (Fe-S) clusters on ISCU (PubMed:29309586). Interacts with the CIA complex member CIAO1 (via LYR motif) (PubMed:29309586).INTERACTION Expressed in lung, brain, stomach, spleen, ovary, testis, liver, muscle and heart.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the HscB family.
created [InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10
crossReference
databaseName UniProt
dbId 148566
description
  • recommendedName: fullName evidence="10"Iron-sulfur cluster co-chaperone protein HscB alternativeName: DnaJ homolog subfamily C member 20 component recommendedName: Iron-sulfur cluster co-chaperone protein HscB, cytoplasmic shortName evidence="9"C-HSC20 /component component recommendedName: Iron-sulfur cluster co-chaperone protein HscB, mitochondrial /component
displayName UniProt:Q8IWL3 HSCB
geneName
  • HSCB
  • DNAJC20
  • HSC20
identifier Q8IWL3
isSequenceChanged false
keyword
  • 3D-structure
  • Chaperone
  • Cytoplasm
  • Metal-binding
  • Mitochondrion
  • Proteomics identification
  • Reference proteome
modified [InstanceEdit:9939033] Weiser, Joel, 2025-02-21
moleculeType Protein
name
  • HSCB
otherIdentifier
  • 11728460_at
  • 150274
  • 16928668
  • 223647_3p_x_at
  • 223647_PM_x_at
  • 223647_x_at
  • 3941624
  • 3941625
  • 3941626
  • 3941627
  • 3941629
  • 3941630
  • 3941631
  • 3941633
  • 3941635
  • 3941638
  • 3941641
  • 3941642
  • 76642_at
  • 8072143
  • 87105_at
  • A_23_P40588
  • A_33_P3309231
  • GE87613
  • GO:0001671
  • GO:0003674
  • GO:0005515
  • GO:0005654
  • GO:0005737
  • GO:0005739
  • GO:0005829
  • GO:0016226
  • GO:0030154
  • GO:0042802
  • GO:0044571
  • GO:0046872
  • GO:0048856
  • GO:0051087
  • GO:0051259
  • GO:0060215
  • GO:0060319
  • GO:0065003
  • GO:0098772
  • HMNXSV003009696
  • HMNXSV003041819
  • ILMN_1771921
  • ILMN_2184789
  • TC22000186.hg
  • g12652532_3p_x_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
referenceTranscript
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • HSC20_HUMAN
  • Q9BWS7
sequenceLength 235
species [Species:48887] Homo sapiens
stId uniprot:Q8IWL3
url http://purl.uniprot.org/uniprot/Q8IWL3

Referrals

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