Product Name	Anti-COX10/APC
Chinese Name	APC标记的细胞色素c氧化酶10抗体
Alias	Heme A farnesyltransferase; Heme O synthase; OTTMUSP00000006085; Protoheme IX farnesyltransferase, mitochondrial precursor; RP23-78H18.1; 2410004F01Rik; AU042636; Cytochrome c oxidase assembly protein; Cytochrome c oxidase subunit X; COX10_HUMAN; Protoheme IX farnesyltransferase, mitochondrial; Heme O synthase.
Research Area	Tumour  Cell biology  Signal transduction  Channel protein  The new supersedes the old  Mitochondrion
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse,  (predicted: Rat, )
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	49kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human COX10
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	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. [provided by RefSeq, Jul 2008] Function: Converts protoheme IX and farnesyl diphosphate to heme O. Subcellular Location: Mitochondrion membrane; Multi-pass membrane protein. DISEASE: Mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]: 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, exercise intolerance, developmental delay, delayed motor development and mental retardation. Some affected individuals manifest a fatal hypertrophic cardiomyopathy resulting in neonatal death. A subset of patients manifest Leigh syndrome. Note=The disease is caused by mutations affecting the gene represented in this entry. Leigh syndrome (LS) [MIM:256000]: An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the UbiA prenyltransferase family. Database links: Entrez Gene: 417329 Chicken Entrez Gene: 511440 Cow Entrez Gene: 1352 Human Entrez Gene: 70383 Mouse Entrez Gene: 363617 Rat Entrez Gene: 553384 Zebrafish Omim: 602125 Human SwissProt: Q12887 Human SwissProt: Q8CFY5 Mouse Unigene: 462278 Human Unigene: 340211 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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