TIMM8A Human

Translocase of Inner Mitochondrial Membrane 8 Homolog A Human Recombinant
Cat. No.
BT24648
Source
Escherichia Coli.
Synonyms
Mitochondrial import inner membrane translocase subunit Tim8 A, TIMM8A, Translocase of Inner Mitochondrial Membrane 8 Homolog A, DDP, DDP1, DFN1, MTS, TIM8, Deafness dystonia protein 1, X-linked deafness dystonia protein.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 90.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

TIMM8A Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 120 amino acids (1-97) and having a molecular mass of 13.4 kDa.
TIMM8A is fused to a 23 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.

Product Specs

Introduction
Translocase of Inner Mitochondrial Membrane 8 Homolog A (TIMM8A) is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondria. This protein acts as a chaperone-like protein, safeguarding hydrophobic precursors from aggregation and guiding them through the mitochondrial intermembrane space. TIMM8A is crucial for transferring beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Defects in TIMM8A are linked to Jensen syndrome. TIMM8A and TIMM13 together form a 70 kDa heterohexamer.
Description
Recombinant human TIMM8A, produced in E. coli, is a single, non-glycosylated polypeptide chain consisting of 120 amino acids (residues 1-97). It has a molecular weight of 13.4 kDa. This protein is fused to a 23 amino acid His-Tag at its N-terminus and is purified using proprietary chromatographic techniques.
Physical Appearance
Clear, colorless, and sterile-filtered solution.
Formulation
The TIMM8A solution is provided at a concentration of 0.25 mg/ml in a buffer consisting of 20mM Tris-HCl (pH 8.0), 0.15M NaCl, 30% glycerol, and 1mM DTT.
Stability
For short-term storage (up to 2-4 weeks), the solution should be stored at 4°C. For extended storage, it is recommended to freeze the solution at -20°C. Adding a carrier protein (0.1% HSA or BSA) is advisable for long-term storage. Repeated freezing and thawing should be avoided.
Purity
The purity of TIMM8A is greater than 90.0% as determined by SDS-PAGE analysis.
Synonyms
Mitochondrial import inner membrane translocase subunit Tim8 A, TIMM8A, Translocase of Inner Mitochondrial Membrane 8 Homolog A, DDP, DDP1, DFN1, MTS, TIM8, Deafness dystonia protein 1, X-linked deafness dystonia protein.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD.

Product Science Overview

Structure and Function

TIMM8A is a mitochondrial intermembrane chaperone that forms a complex with another protein called TIMM13 . This complex, often referred to as the TIM8-TIM13 complex, is essential for the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane . The TIM8-TIM13 complex also facilitates the transfer of beta-barrel precursors from the TOM complex (Translocase of the Outer Membrane) to the SAM complex (Sorting and Assembly Machinery) of the outer membrane .

Genetic and Clinical Significance

Mutations in the TIMM8A gene are associated with several genetic disorders, including Mohr-Tranebjaerg syndrome (MTS), also known as Deafness-Dystonia Syndrome (DDS) . This X-linked recessive neurodegenerative disorder is characterized by progressive hearing loss, dystonia, and other neurological symptoms . Additionally, defects in the TIMM8A gene can cause Jensen syndrome, an X-linked disease marked by opticoacoustic nerve atrophy and muscle weakness .

Research and Applications

The recombinant form of TIMM8A is used in various research applications to study its role in mitochondrial function and its involvement in mitochondrial diseases . Understanding the mechanisms by which TIMM8A and its associated complexes operate can provide insights into the development of therapeutic strategies for mitochondrial disorders.

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