chchd4

Mitochondrial intermembrane space import and assembly protein 40

146 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria.

15.755 kDa

MSYCKQEGKDCIIFVTKEDHEAPSNAELVEDDPNDPYEDHGLILPNGDINWNCPCLGGMASGPCGQQFKDAFSCFHYSKEEIKGSDCVENFRGMQECMQKYPELYPQEDDNDSAPSGGANTAPTDSLPASSTDSTAAAATENPATS

CHCHD4

Mitochondrial intermembrane space import and assembly protein 40

142 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364, PubMed:23676665). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS (PubMed:16185709, PubMed:19182799, PubMed:21059946, PubMed:23676665). Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system (PubMed:23186364). Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake (PubMed:26387864).

15.996 kDa

MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGNINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEEIKGSDCVDQFRAMQECMQKYPDLYPQEDEDEEEEREKKPAEQAEETAPIEATATKEEEGSS

Chchd4

Mitochondrial intermembrane space import and assembly protein 40

139 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system. Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake.

15.525 kDa

MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEDIKGSDCIDQFRAMQECMQKYPDLYPQDEEEEEEAKPVEPVEETADTKVSAAKEQGTSS

Chchd4

Mitochondrial intermembrane space import and assembly protein 40

139 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system. Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake.

15.467 kDa

MSYCRQEGKDRIIFVTKEDHETPSSAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSTEDIKGSDCIDQFRAMQECMQKYPDLYPQDEEEEEEAKPVEPVEETADTKASAAKEQGASS

chchd4

Mitochondrial intermembrane space import and assembly protein 40

139 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria.

15.712 kDa

MSYCRQEGKDKIIFVTKEDHETPSSAELIADDPNDPYEDHGLILPNGDINWNCPCLGGMASGPCGEQFKSAFSCFHYSQEEIKGSDCLDQFRAMQECMQKYPDIYPQEDDEDEAEKEKQNKEAEAFSTETSDTKEESSS

CHCHD4

Mitochondrial intermembrane space import and assembly protein 40

137 amino acids

Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17, COX19 and MICU1. Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria. The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS. Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system. Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake.

15.173 kDa

MAYCRQEGKDRIIFVTKEDHETPSNAELVADDPNDPYEEHGLILPNGDINWNCPCLGGMASGPCGEQFKAAFSCFHYSKEDVKGSDCVDQFRAMQECMQKYPDLYPQEEEEEEEQPADPLPEAASEASATKEAAASS