4.4.1.17: Holocytochrome-c synthase

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For detailed information about Holocytochrome-c synthase, go to the full flat file.

Reaction

Synonyms

CCHL, ccsA1, cyc2p, CYC3, cytochrome c heme lyase, Cytochrome c heme-lyase, Cytochrome c synthase, HCC synthase, HCCH, HCCS, heme lyase, holocytochrome c synthase, Holocytochrome c synthetase, Holocytochrome c-type synthase, Holocytochrome-C synthase, Holocytochrome-C-type synthase, Synthetase, holocytochrome c, System III

ECTree

     4 Lyases

         4.4 Carbon-sulfur lyases

             4.4.1 Carbon-sulfur lyases (only sub-subclass identified to date)

                4.4.1.17 Holocytochrome-c synthase

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Results	in table
6	Activating Compound
4729	AA Sequence
4	Application
1	CAS Registry Number
18	Cloned(Commentary)
2	Cofactor
505	Disease/ Diagnostics
8	General Information
4	Inhibitors
1	Ki Value [mM]
1	KM Value [mM]
26	Localization
4	Molecular Weight [Da]
9	Natural Substrates/ Products (Substrates)
35	Organism
4	Pathway
1	pH Optimum
1	pH Range
39	Protein Variants
7	Purification (Commentary)
1	Reaction
1	Reaction Type
34	Reference
3	Source Tissue
3	Specific Activity [micromol/min/mg]
50	Substrates and Products (Substrate)
2	Subunits
32	Synonyms
1	Systematic Name
3	Temperature Optimum [°C]

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Application on EC 4.4.1.17 - Holocytochrome-c synthase

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APPLICATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

medicine

2 entries

molecular biology

Saccharomyces cerevisiae

P06182

system III, cytochrome c heme lyase, is an enzyme found in the mitochondria of many eukaryotes, which is used for heterologous expression of mitochondrial holocytochromes c

730322

synthesis

Homo sapiens

P53701

mutants E159A and W118A display enhanced release of cytochrome c from the active site. The mutant allows for synthesis of cytochrome c variants such as cyt c H19M (bis-Met), cyt c M81H (bis-His), cyt c M81A (His/OH), cyt c C15S, which exhibit proper folding

749183

medicine

Homo sapiens

-

identification of mutations R217C and DELTA197-268 in female patients with microphthalmia with linear skin defects syndrome. In contrast to wild-type, mutant proteins are unable to complement a Saccharomyces cerevisiae mutant deficient in the yeast enzyme ortholog. Mutation results in disturbance of both oxidative phosphorylation and the balance between apoptosis and necrosis. Upon expression in CHO-K1 cells, mutant DELTA197-268 protein fails to be sorted to mitochondria

677440

medicine

Homo sapiens

P53701

patient with deletion of the HCCS gene, diagnosis of a microphthalmia with linaer skin defects syndrome. Patient showed bilateral microphthalmia with optic atrophy, a congenital cataract, linear vascular lesions on the cheeks, neck and nose, apparently small, cupped poorly formed ears, anteverted nares, small areolae, a prominent xiphoid, an apparently small phallus, and right cryptorchidism. He had a patent ductus arteriosus, a patent foramen ovale and severe pulmonary hypertension attributed to pulmonary hypoplasia. Head ultrasound showed bilateral ventriculomegaly and agenesis of the corpus callosum. He also had poor tone and diminished reflexes on neurological examination. Patient died at 4 days of age

713714