Metabolism Antibodies 2

Anti-COX10 Antibody (CAB7547)

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SKU:
CAB7547
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-COX10 Antibody
Antibody SKU:CAB7547
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-160 of human COX10 (NP_001294.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:Mouse esophagus
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-160 of human COX10 (NP_001294.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MAAS PHTL SSRL LTGC VGGS VWYL ERRT IQDS PHKF LHLL RNVN KQWI TFQH FSFL KRMY VTQL NRSH NQQV RPKP EPVA SPFL EKTS SGQA KAEI YEMR PLSP PSLS LSRK PNEK ELIE LEPD SVIE DSID VGKE TKEE KRWK EMKL QVYD LPGI LARL
Gene ID:1352
Uniprot:Q12887
Cellular Location:Mitochondrion membrane, Multi-pass membrane protein
Calculated MW:27kDa/48kDa
Observed MW:49kDa
Synonyms:COX10
Background:Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion.
UniProt Protein Function:COX10: Converts protoheme IX and farnesyl diphosphate to heme O. Defects in COX10 are a cause of mitochondrial complex IV deficiency (MT-C4D); 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. Defects in COX10 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Belongs to the UbiA prenyltransferase family.
UniProt Protein Details:

Protein type:Membrane protein, integral; Membrane protein, multi-pass; Transferase; Energy Metabolism - oxidative phosphorylation; EC 2.5.1.-; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll

Chromosomal Location of Human Ortholog: 17p12

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function:cytochrome-c oxidase activity; farnesyltranstransferase activity; protoheme IX farnesyltransferase activity

Biological Process: cellular respiration; heme a biosynthetic process; heme biosynthetic process; mitochondrial electron transport, cytochrome c to oxygen; respiratory chain complex IV assembly

Disease: Leigh Syndrome; Mitochondrial Complex Iv Deficiency

NCBI Summary:Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lysine for an asparagine (N204K), is identified to be responsible for cytochrome c oxidase deficiency. In addition, this gene is disrupted in patients with CMT1A (Charcot-Marie-Tooth type 1A) duplication and with HNPP (hereditary neuropathy with liability to pressure palsies) deletion. [provided by RefSeq, Jul 2008]
UniProt Code:Q12887
NCBI GenInfo Identifier:292495084
NCBI Gene ID:1352
NCBI Accession:Q12887.3
UniProt Secondary Accession:Q12887,O15334, Q969F7, B2R6U5, B4DJ50,
UniProt Related Accession:Q12887
Molecular Weight:27,594 Da
NCBI Full Name:Protoheme IX farnesyltransferase, mitochondrial
NCBI Synonym Full Names:COX10, heme A:farnesyltransferase cytochrome c oxidase assembly factor
NCBI Official Symbol:COX10  
NCBI Protein Information:protoheme IX farnesyltransferase, mitochondrial
UniProt Protein Name:Protoheme IX farnesyltransferase, mitochondrial
UniProt Synonym Protein Names:Heme O synthase
Protein Family:Probable protoheme IX farnesyltransferase
UniProt Gene Name:COX10  
UniProt Entry Name:COX10_HUMAN
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