TIMM8A/DDP anticorps (AA 14-97)

N° du produit ABIN7600039

Cet anticorps anti-TIMM8A/DDP est un anticorps Lapin Polyclonal détectant TIMM8A/DDP dans WB, ELISA, IHC, IF et ICC. Adapté pour Humain, Souris, Rat et Singe.

Aperçu rapide pour TIMM8A/DDP anticorps (AA 14-97) (ABIN7600039)

Antigène: TIMM8A/DDP (TIMM8A) (Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))

Reactivité: Humain, Souris, Rat, Singe

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp TIMM8A/DDP est non-conjugé

Application: Western Blotting (WB), ELISA, Immunohistochemistry (IHC), Immunofluorescence (IF), Immunocytochemistry (ICC)

Détail du produit anti-TIMM8A/DDP anticorps

Épitope: AA 14-97

Fonction: Anti-TIMM8A/DDP Antibody Picoband®

Réactivité croisée (Details): No cross-reactivity with other proteins.

Attributs du produit: 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.

Purification: Immunogen affinity purified.

Immunogène: E.coli-derived human TIMM8A/DDP recombinant protein (Position: A14-D97).

Isotype: IgG

Information d'application

Indications d'application: 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.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Concentration: 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Détails sur TIMM8A/DDP

Antigène: TIMM8A/DDP (TIMM8A) (Translocase of Inner Mitochondrial Membrane 8A (TIMM8A))

Autre désignation: TIMM8A

Sujet:

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.

Poids moléculaire: 13-14 kDa

ID gène: 1678

UniProt: O60220