Acts as a co-chaperone in iron-sulfur cluster assembly in mitochondria . 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 . Plays an essential role in hematopoiesis (By similarity).
Acts as a co-chaperone in iron-sulfur cluster assembly in the cytoplasm . 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 .
Subcellular locations: Cytoplasm
Subcellular locations: Mitochondrion
Expressed in lung, brain, stomach, spleen, ovary, testis, liver, muscle and heart.