TIMM8A Mouse Monoclonal Antibody [Clone ID: LBI5C8]

Product Specifications

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. [provided by RefSeq, Mar 2009]

Overview

Carrier-free (BSA/glycerol-free) TIMM8A mouse monoclonal antibody, clone LBI5C8

Synonyms

DDP; DDP1; DFN1; MTS; TIM8

Gene Name

Homo sapiens translocase of inner mitochondrial membrane 8A (TIMM8A), transcript variant 1, mRNA; nuclear gene for mitochondrial product.

Gene ID

1678

UniProt

O60220

Conjugation

Unconjugated

Dilution

WB 1:500~2000, IHC 1:500

Form

Lyophilized powder (original buffer 1X PBS, pH 7.3, 8% trehalose)

Buffer

Lyophilized powder (original buffer 1X PBS, pH 7.3, 8% trehalose)

Function

Druggable Genome

Storage Conditions

Stable for 1 year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and opening the cap. Aliquot will be stable at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.