Anti-AOX3抗体Cytochrome C Oxidase subunits I, II and III form the functional core of the enzyme complex.

产品编号 yb-3934R
英文名称 Anti-AOX3抗体
中文名称 *氧化酶亚基3抗体
别    名 Cytochrome c oxidase polypeptide III; Cytochrome c oxidase subunit 3; COIII; COX3; COX-3; COXIII; MT CO3; MTCO3; COX3_HUMAN.
Anti-AOX3抗体 
说 明 书 0.2ml  
研究领域 肿瘤  细胞生物  免疫学  神经生物学  信号转导  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 30kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human COX3
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
Cytochrome C Oxidase subunits I, II and III form the functional core of the enzyme complex.
Defects in Cytochrome C Oxidase subunits III are a cause of Leber hereditary optic neuropathy (LHON) and cytochrome c oxidase deficiency (COX deficiency). Defects in Cytochrome C Oxidase subunits III are also found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome and recurrent myoglobinuria.

Function:
Subunits I, II and III form the functional core of the enzyme complex.

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein (By similarity).

DISEASE:
Leber hereditary optic neuropathy (LHON) [MIM:535000]: 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=The disease is caused by mutations affecting the gene represented in this entry. 
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. 
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. Note=The gene represented in this entry may be involved in disease pathogenesis. 

Similarity:
Belongs to the cytochrome c oxidase subunit 3 family.

Database links:
UniProtKB/Swiss-Prot: P00414.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.