TIMM8A/DDP 抗体 (AA 14-97)
Quick Overview for TIMM8A/DDP 抗体 (AA 14-97) (ABIN7600039)
抗原
See all TIMM8A/DDP (TIMM8A) 抗体适用
宿主
克隆类型
标记
应用范围
-
-
抗原表位
- AA 14-97
-
原理
- Anti-TIMM8A/DDP Antibody Picoband®
-
交叉反应 (详细)
- No cross-reactivity with other proteins.
-
产品特性
- Anti-TIMM8A/DDP Antibody Picoband® (ABIN7600039). Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Monkey, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
-
纯化方法
- Immunogen affinity purified.
-
免疫原
- E.coli-derived human TIMM8A/DDP recombinant protein (Position: A14-D97).
-
亚型
- IgG
-
-
-
-
应用备注
-
Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat, Monkey
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/mL, Human, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Aguirre, L. A., del Castillo, I., Macaya, A., Meda, C., Villamar, M., Moreno-Pelayo, M. A., Moreno, F. A novel mutation in the gene encoding TIMM8a, a component of the mitochondrial protein translocase complexes, in a Spanish familial case of deafness-dystonia (Mohr-Tranebjaerg) syndrome. Am. J. Med. Genet. 140A: 392-397, 2006. 2. Ezquerra, M., Campdelacreu, J., Munoz, E., Tolosa, E., Marti, M. J. A novel intronic mutation in the DDP1 gene in a family with X-linked dystonia-deafness syndrome. Arch. Neurol. 62: 306-308, 2005. 3. Hofmann, S., Rothbauer, U., Muhlenbein, N., Neupert, W., Gerbitz, K.-D., Brunner, M., Bauer, M. F. The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1-TIM13 complexes in the mitochondrial intermembrane space. J. Biol. Chem. 277: 23287-23293, 2002. -
限制
- 仅限研究用
-
-
-
状态
- Lyophilized
-
溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
-
浓度
- 500 μg/mL
-
缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.
-
储存液
- Sodium azide
-
注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
储存条件
- 4 °C,-20 °C
-
储存方法
-
Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
-
-
- TIMM8A/DDP (TIMM8A) (Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))
-
别名
- TIMM8A
-
背景
-
Synonyms: Cadherin-4, Retinal cadherin, R-CAD, R-cadherin, CDH4
Tissue Specificity: Expressed mainly in brain but also found in other tissues.
Background: Mitochondrial import inner membrane translocase subunit Tim8 A, also known as Deafness-dystonia peptide or protein is an enzyme that in humans is encoded by the TIMM8A gene. This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome, an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
-
分子量
- 13-14 kDa
-
基因ID
- 1678
-
UniProt
- O60220
抗原
-