protein

Recombinant Human Cytochrome c oxidase subunit 1 (MT-CO1)

MBS961415

1 mg (E Coli Derived

1375 USD

Recombinant Protein

Recombinant Human Cytochrome c oxidase subunit 1 (MT-CO1)

Cytochrome c oxidase subunit 1 (MT-CO1)

Recombinant Cytochrome c oxidase subunit 1 (MT-CO1); Cytochrome c oxidase subunit 1 EC= 1.9.3.1; Cytochrome c oxidase polypeptide I

cytochrome c oxidase subunit I; Cytochrome c oxidase subunit 1; cytochrome c oxidase subunit I; mitochondrially encoded cytochrome c oxidase I; Cytochrome c oxidase polypeptide I

MT-CO1; COI, COXI, MTCO1

MT-COI; MT-CO1; COI; MTCO1; COX1; COI; COXI; MTCO1

COX1_HUMAN

E Coli or Yeast

1-513

513

MFADRWLFSTNHKDIGTLYLLFGAWAGVLGTALSLLIRA
ELGQPGNLLGNDHIYNVIVTAHAFVMIFFMVMPIMIGGF
GNWLVPLMIGAPDMAFPRMNNMSFWLLPPSLLLLLASAM
VEAGAGTGWTVYPPLAGNYSHPGASVDLTIFSLHLAGVS
SILGAINFITTIINMKPPAMTQYQTPLFVWSVLITAVLL
LLSLPVLAAGITMLLTDRNLNTTFFDPAGGGDPILYQHL
FWFFGHPEVYILILPGFGMISHIVTYYSGKKEPFGYMGM
VWAMMSIGFLGFIVWAHHMFTVGMDVDTRAYFTSATMII
AIPTGVKVFSWLATLHGSNMKWSAAVLWALGFIFLFTVG
GLTGIVLANSSLDIVLHDTYYVVAHFHYVLSMGAVFAIM
GGFIHWFPLFSGYTLDQTYAKIHFTIMFIGVNLTFFPQH
FLGLSGMPRRYSDYPDAYTTWNILSSVGSFISLTAVMLM
IFMIWEAFASKRKVLMVEEPSMNLEWLYGCPPPYHTFEE
PVYMKS

>90%

This item requires custom production and lead time is between 5-9 weeks. We can custom produce according to your specifications.

Store at -20 degree C. For extended storage, store at -20 or -80 degree C.

Tag Information: His tagged (Host tag may vary. Please inquire for specific tag information). Species: Homo sapiens (Human)

Storage Buffer: PBS pH 7.4, 50% glycerol

251831109

YP_003024028.1

NC_012920.1

P00395

57,041 Da

Alzheimer's Disease Pathway 83097!!Alzheimer's Disease Pathway 509!!Cardiac Muscle Contraction Pathway 93344!!Cardiac Muscle Contraction Pathway 93992!!Cytochrome C Oxidase Pathway 413434!!Cytochrome C Oxidase Pathway 468345!!Electron Transport Chain Pathway 198860!!Huntington's Disease Pathway 83100!!Huntington's Disease Pathway 512!!Metabolism Pathway 477135

Function: Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B. Catalytic activity: 4 ferrocytochrome c + O2 + 4 H+ = 4 ferricytochrome c + 2 H2O. Pathway: Energy metabolism; oxidative phosphorylation. Subcellular location: Mitochondrion inner membrane; Multi-pass membrane protein. Involvement in disease: Defects in MT-CO1 are a cause of Leber hereditary optic neuropathy (LHON) [. MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes. Ref.10Note=MT-CO1 may play a role in the pathogenesis of acquired idiopathic sideroblastic anemia, a disease characterized by inadequate formation of heme and excessive accumulation of iron in mitochondria. Mitochondrial iron overload may be attributable to mutations of mitochondrial DNA because these can cause respiratory chain dysfunction, thereby impairing reduction of ferric iron to ferrous iron. The reduced form of iron is essential to the last step of mitochondrial heme biosynthesis.Defects in MT-CO1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [. MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Ref.14 Ref.15Defects in MT-CO1 are associated with recurrent myoglobinuria mitochondrial (RM-MT) [. MIM:550500]. Recurrent myoglobinuria is characterized by recurrent attacks of rhabdomyolysis (necrosis or disintegration of skeletal muscle) associated with muscle pain and weakness, and followed by excretion of myoglobin in the urine. Ref.13Defects in MT-CO1 are a cause of deafness sensorineural mitochondrial (DFNM) [. MIM:500008]. DFNM is a form of non-syndromic deafness with maternal inheritance. Affected individuals manifest progressive, postlingual, sensorineural hearing loss involving high frequencies. Ref.8Defects in MT-CO1 are a cause of colorectal cancer (CRC) [. MIM:114500]. Ref.16 Ref.17. Sequence similarities: Belongs to the heme-copper respiratory oxidase family.

1 mg (E Coli Derived)

1375 USD