Mouse anti-Human COX10 Polyclonal Antibody | anti-COX10 antibody

COX10 (Protoheme IX Farnesyltransferase, Mitochondrial, Heme O Synthase)

Cat. Num. AAA6001468

• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: COX10; Polyclonal Antibody; COX10 (Protoheme IX Farnesyltransferase; Mitochondrial; Heme O Synthase); Anti -COX10 (Protoheme IX Farnesyltransferase; anti-COX10 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human COX10.
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2.
• Sequence: MAASPHTLSSRLLTGCVGGSVWYLERRTIQDSPHKFLHLLRNVNKQWITFQHFSFLKRMYVTQLNRSHNQQVRPKPEPVASPFLEKTSSGQAKAEIYEMRPLSPPSLSLSRKPNEKELIELEPDSVIEDSIDVGKETKEEKRWKEMKLQVYDLPGILAQLSKIKLTALVVSTTAAGFALAPGPFDWPCFLLTSVGTGLASCAANSINQFFEVPFDSNMNRTKNRPLVRGQISPLLAVSFATCCAVPGVAILTLGVNPLTGALGLFNIFLYTCCYTPLKRISIANTWVGAVVGAIPPVMGWTAATGSLDAGAFLLGGILYSWQFPHFNALSWGLREDYSRGGYCMMSVTHPGLCRRVALRHCLALLVLSAAAPVLDITTWTFPIMALPINAYISYLGFRFYVDADRRSSRRLFFCSLWHLPLLLLLMLTCKRPSGGGDAGPPPS

• Applicable Applications for anti-COX10 antibody: Western Blot (WB)
• Application Notes: Suitable for use in Western Blot.
• Immunogen: Full length human COX10, aa1-443 (AAH06394).
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of COX10 expression in transfected 293T cell line by COX10 polyclonal antibody. Lane 1: COX10 transfected lysate (48.73kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-COX10 antibody

Converts protoheme IX and farnesyl diphosphate to heme O.

Product Categories/Family for anti-COX10 antibody

Antibodies; Abs to Cytochrome

NCBI and Uniprot Product Information

• NCBI GI #: 119610363
• NCBI GeneID: 1352
• UniProt Accession #: Q12887; O15334; Q969F7; B2R6U5
• Molecular Weight: 48,910 Da
• NCBI Official Full Name: COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast), isoform CRA_b
• NCBI Official Synonym Full Names: cytochrome c oxidase assembly homolog 10 (yeast)
• NCBI Official Symbol: COX10
• NCBI Protein Information: protoheme IX farnesyltransferase, mitochondrial; heme O synthase; heme A: farnesyltransferase; cytochrome c oxidase subunit X; cytochrome c oxidase assembly protein; COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase
• UniProt Protein Name: Protoheme IX farnesyltransferase, mitochondrial
• Protein Family: Probable protoheme IX farnesyltransferase
• UniProt Gene Name: COX10
• UniProt Entry Name: COX10_HUMAN

NCBI Description

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 Description

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.

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

Chromosomal Location of Human Ortholog: 17p12

Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane

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

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

Disease: Leigh Syndrome; Mitochondrial Complex Iv Deficiency