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Rabbit Anti-COX10  antibody (bs-23098R)  
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產品編號 bs-23098R
英文名稱 Rabbit Anti-COX10  antibody
中文名稱 細胞色素c氧化酶10抗體
別    名 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.  
研究領域 腫瘤  細胞生物  信號轉導  通道蛋白  新陳代謝  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse (predicted: Rat)
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 49kDa
細胞定位 細胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COX10: 161-260/443 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 Preservative: 0.02% Proclin300, Constituents: 1% BSA, 0.01M PBS, pH7.4.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q12887

Gene ID:
1352

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



產品圖片
Sample: HL-60 Cell (Human) Lysate at 40 ug Primary: Anti-COX10 (bs-23098R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 49 kD Observed band size: 50 kD
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (COX10) Polyclonal Antibody, Unconjugated (bs-23098R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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