产品名称：Anti-ANT-1/FITC抗体

Anti-ANT-1/FITC抗体

产品编号YB-6794R-FITC

英文名称Anti-ANT-1/FITC

中文名称FITC标记的腺嘌呤核苷酸转运蛋白1抗体

别    名heart/skeletal muscle isoform T1; Adenine nucleotide translocator 1 (skeletal muscle); Adenine nucleotide translocator 1; ADP; ADP ATP carrier protein 1; ADP ATP carrier protein heart/skeletal muscle isoform T1; ADP/ATP translocase 1; ADT1_HUMAN; ANT 1; ANT; ANT1; ATP carrier protein 1; ATP carrier protein; MSA02; PEO2; PEO3; SLC25A4; Solute carrier family 25 member 4; T1 antibody.

Anti-ANT-1/FITC抗体    

说 明 书100ul  

研究领域细胞生物  **学  神经生物学  信号转导  细胞凋亡  细胞类型标志物  G蛋白信号  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep,

产品应用IF=1:50-200  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量33kDa

细胞定位细胞膜

性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from human ATP carrier protein 1/Adenine Nucleotide Translocase 1

亚    型IgG

纯化方法affinity purified by Protein A

储 存 液Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

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.

Anti-ANT-1/FITC抗体

产品介绍background:

Defects in SLC25A4 are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 2 (PEOA2) [MIM:609283]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.

Subunit:

Found in a complex with ARL2, ARL2BP and SLC25A4. Interacts with ARL2BP (By similarity). Homodimer. Interacts with HIV-1 Vpr.

Subcellular Location:

Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE:

Defects in SLC25A4 are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant type 2 (PEOA2) [MIM:609283]. Progressive external ophthalmoplegia is characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, Anti-ANT-1/FITC抗体which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism.

Anti-ANT-1/FITC抗体Similarity:

Belongs to the mitochondrial carrier family.

Contains 3 Solcar repeats.

Database links:

Entrez Gene: 291 Human

Omim: 103220 Human

SwissProt: P12235 Human

Unigene: 246506 Human

Important Note:

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