DISEASES

Disease-gene associations mined from literature

Literature on HSCB

HSCB [ENSP00000216027]

Iron-sulfur cluster co-chaperone protein HscB, mitochondrial; Acts as a co-chaperone in iron-sulfur cluster assembly in both mitochondria and the cytoplasm . 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 . 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 . 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 . This facilitates iron-sulfur cluster insertion into a number of cytoplasmic and nuclear proteins including POLD1, ELP3, DPYD and PPAT .

Synonyms:  HSCB,  A0A384NYJ4,  B0QYH2,  F8WBY7,  Q8IWL3 ...

Linkouts:  STRING  Pharos  UniProt  OMIM