TIMM8A/DDP anticorps

N° du produit ABIN6572020

Cet anticorps Lapin Polyclonal détecte spécifiquement TIMM8A/DDP dans WB. Il présente une réactivité envers Humain et Souris.

Aperçu rapide pour TIMM8A/DDP anticorps (ABIN6572020)

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

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp TIMM8A/DDP est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-TIMM8A/DDP anticorps

Purification: Affinity purification

Immunogène: Recombinant protein of human TIMM8A

Isotype: IgG

Information d'application

Indications d'application: WB 1:500 - 1:2000

Restrictions: For Research Use only

Stockage

Concentration: 1 mg/mL

Buffer: Buffer: PBS with 0.02 % sodium azide, 50 % glycerol,  pH 7.3.

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: -20 °C

Stockage commentaire: Store at -20°C. Avoid freeze / thaw cycles.

Détails sur TIMM8A/DDP

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

Autre désignation: TIMM8A

Sujet:

Synonyms: DDP 1,DDP,DDP1,Deafness dystonia protein 1,Deafness/dystonia peptide,DFN 1,DFN1,MGC12262,Mitochondrial import inner membrane translocase subunit Tim8 A,MTS,TIM 8A,TIM8,TIM8A,TIM8A,TIMM 8A,timm8a,Translocase of inner mitochondrial membrane 8 homolog A,X linked deafness dystonia protein,X-linked deafness dystonia protein

Background: 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:

Observed_MW: 11kDa

Calculated_MW: 10kDa

ID gène: 1678

UniProt: O60220