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Rabbit Anti-ANT-1/Gold Conjugated antibody (bs-6794R-Gold)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產品編號 bs-6794R-Gold
英文名稱 Rabbit Anti-ANT-1/Gold Conjugated antibody
中文名稱 膠體金標記的腺嘌呤核苷酸轉運蛋白1抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 細胞生物  免疫學  神經生物學  信號轉導  細胞凋亡  細胞類型標志物  G蛋白信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep, )
產品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 33kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ATP carrier protein 1/Adenine Nucleotide Translocase 1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產品介紹 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, 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.

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.
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