CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial

CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial
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Topology in Mitochondrial inner membrane
Topologyintermembrane space
mitochondrial matrix side
CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial » Complex III subunit 4;Complex III subunit IV;Cytochrome b-c1 complex subunit 4;Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit;Cytochrome c-1;
Hydrophobic Thickness 28.2 ± 2.8 Å
Tilt Angle 14 ± 3°
ΔGtransfer -24.1 kcal/mol
ΔGfold -14.9 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, Reactome, HMDB
Topology Out
TM Segments 288-307 (281-309)
Pathways

Alzheimer's disease (KEGG)

Cardiac muscle contraction (KEGG)

Huntington's disease (KEGG)

Nitrogen metabolism (KEGG)

Oxidative phosphorylation (KEGG)

Parkinson's disease (KEGG)

Two-component system (KEGG)

PDB none
OPM 1l0l (BOVIN), 1pp9 (BOVIN), 1zrt (RHOCA), 2a06 (BOVIN), 2fyu (BOVIN), 2qjy (RHOSH), 2ybb (BOVIN), 2yiu (PARDE), 3cx5 (YEAST), 5xte
Complexes

CY1:CYB:UCRI_RHOSH

UCRI:CYB:CY1_PARDE

CYB:UCRI:CY1_RHOCA

HVM60:QCR6:QCR7:CYB:KV5AE:CY1:QCR1:QCR2:UCRI:QCR8:QCR9_YEAST

CY1:QCR6:QCR7:QCR9:CYB:QCR8:UCRI:QCR2:QCR1_BOVIN

QCR10:CY1:QCR6:QCR7:QCR9:CYB:QCR8:UCRI:QCR2:QCR1_BOVIN

CYC1:QCR6:QCR7:CYB:CY1:QCR1:QCR2:UCRI:QCR8:QCR9_YEAST

QCR10:CYC:COX1:COX3:COX41:COX5A:COX5B:CX6B1:COX7C:COX6C:CX7A1:CX6A2:COX8B:COX7B:COX2:CY1:QCR6:QCR7:QCR9:CYB:QCR8:UCRI:QCR2:QCR1:NQO6:NQO5:NQO4:NQO2:NQO1:NQO3:NQO9:NQO15_BOVIN

Interactions

PYRD, Complex: PYRD:CY1, PubMed

QCR10, Complex: CY1:CYB:QCR10:QCR1:QCR2:QCR6:QCR7:QCR8:QCR9:UCRI, PDBID: 1be3

UCRI, Complex: CYB:UCRI:CY1:QCR6:QCR7:QCR9:QCR8:UCRI:QCR2:QCR1, PDBID: 1BCC

Domains

AA: 96-314, PDBID: 1BCC, Subunit D, Seq Identity:92%, Cytochrome C1 family

UniProt annotation for CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial
FUNCTION: This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain.

SUBUNIT: The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).

DISEASE: Mitochondrial complex III deficiency, nuclear 6 (MC3DN6) OMIM: An autosomal recessive disorder caused by mitochondrial dysfunction. It is characterized by onset in early childhood of episodic acute lactic acidosis, ketoacidosis, and insulin-responsive hyperglycemia, usually associated with infection. Laboratory studies show decreased activity of mitochondrial complex III. Psychomotor development is normal. mutations affecting the gene represented in this entry.

UniProt features for CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial
TRANSIT 1 84 Mitochondrion.
CHAIN 85 325 Cytochrome c1, heme protein, mitochondrial.
DOMAIN 108 209 Cytochrome c.
Amino Acid Sequence for CY1_HUMAN » Cytochrome c1, heme protein, mitochondrial
MAAAAASLRG VVLGPRGAGL PGARARGLLC SARPGQLPLR TPQAVALSSK SGLSRGRKVM LSALGMLAAG GAGLAVALHS AVSASDLELH PPSYPWSHRG LLSSLDHTSI RRGFQVYKQV CASCHSMDFV AYRHLVGVCY TEDEAKELAA EVEVQDGPNE DGEMFMRPGK LFDYFPKPYP NSEAARAANN GALPPDLSYI VRARHGGEDY VFSLLTGYCE PPTGVSLREG LYFNPYFPGQ AIAMAPPIYT DVLEFDDGTP ATMSQIAKDV CTFLRWASEP EHDHRKRMGL KMLMMMALLV PLVYTIKRHK WSVLKSRKLA YRPPK