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COX1/MTCO1 Rabbit pAb (bs-3953R)  
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產品編號 bs-3953R
英文名稱 COX1/MTCO1 Rabbit pAb
中文名稱 細胞色素c氧化酶1抗體
別    名 COX1_HUMAN; Cytochrome c oxidase subunit 1; EC:7.1.1.9; Cytochrome c oxidase polypeptide I; MT-CO1; COI; COXI; COX I; MTCO1;  
Specific References  (5)     |     bs-3953R has been referenced in 5 publications.
[IF=6.684] Ying Yang. et al. ING2 Controls Mitochondrial Respiration via Modulating MRPL12 Ubiquitination in Renal Tubular Epithelial Cells. Front Cell Dev Biol. 2021; 9: 700195  WB ;  Mouse.  
[IF=6.17] Xia Gu. et al. Transcription of MRPL12 regulated by Nrf2 contributes to the mitochondrial dysfunction in diabetic kidney disease. Free Radical Bio Med. 2021 Feb;164:329  WB ;  Human.  
[IF=3.998] Renata R. Braga. et al. Exercise alters the mitochondrial proteostasis and induces the mitonuclear imbalance and UPR mt in the hypothalamus of mice. Sci Rep-Uk. 2021 Feb;11(1):1-13  IF ;  Mouse.  
[IF=3.309] He R et al. MiR-1a-3p mitigates isoproterenol-induced heart failure by enhancing the expression of mitochondrial ND1 and COX1.Exp Cell Res. 2019 May 1;378(1):87-97.  IHC-P&WB ;  Mouse.  
[IF=1.35] Hui, Yan, et al. "Resveratrol improves mitochondrial function in the remnant kidney from 5/6 nephrectomized rats." Acta Histochemica (2017).  WB ;  Rat.  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  激酶和磷酸酶  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse,Rat (predicted: Human,Rabbit,Pig,Cow,Chicken,Dog,Horse)
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 57 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human COX1: 401-500/513 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
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 Contributes to cytochrome-c oxidase activity. Predicted to be involved in electron transport coupled proton transport and mitochondrial electron transport, cytochrome c to oxygen. Part of mitochondrial respiratory chain complex III and mitochondrial respiratory chain complex IV. [provided by Alliance of Genome Resources, Apr 2022]

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.

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.

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.
Note=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.
Defects 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.
Defects 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.
Defects in MT-CO1 are a cause of colorectal cancer (CRC) [MIM:114500].

Similarity:
Belongs to the heme-copper respiratory oxidase family.

SWISS:
P00395

Gene ID:
4512

Database links:

Entrez Gene: 281919 Cow

Entrez Gene: 4512 Human

Entrez Gene: 17708 Mouse

Entrez Gene: 26195 Rat

Entrez Gene: 140539 Zebrafish

SwissProt: P00396 Cow

SwissProt: P00395 Human

SwissProt: P00397 Mouse

SwissProt: P05503 Rat

SwissProt: Q9MIY8 Zebrafish



產品圖片
Sample: Lane 1: Mouse Heart Lysates Lane 2: Mouse Skeletal muscle Lysates Lane 3: Rat Heart Lysates Lane 4: Rat Muscle Lysates Primary: Anti-COX1MTCO1 (bs-3953R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 57kDa Observed band size: 45kDa
Paraformaldehyde-fixed, paraffin embedded (Rat kidney); 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 (COX1) Polyclonal Antibody, Unconjugated (bs-3953R) at 1:500 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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