Recombinant Human TIMM8A His Protein

SDS-Page: Recombinant Human TIMM8A Protein [NBP2-22882]

• Reactivity: Hu
• Applications: PAGE
• Concentration: 0.25 mg/ml

Recombinant Human TIMM8A His Protein Summary

• Description: A recombinant protein with a N-Terminal His-tag and corresponding to the amino acids 1-97 of Human TIMM8A
  
  

  Source: E.coli

  Amino Acid Sequence: MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD

• Source: E. coli
• Protein/Peptide Type: Recombinant Protein
• Gene: TIMM8A
• Purity: >90%, by SDS-PAGE

Applications/Dilutions

• Dilutions:
  • SDS-Page
• Theoretical MW: 13.4 kDa.
  Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Packaging, Storage & Formulations

• Storage: Store at 4C short term. Aliquot and store at -20C long term. Avoid freeze-thaw cycles.
• Buffer: 20 mM Tris-HCl buffer (pH 8.0), 0.15 M NaCl, 30% glycerol, 1 mM DTT
• Preservative: No Preservative
• Concentration: 0.25 mg/ml
• Purity: >90%, by SDS-PAGE

Alternate Names for Recombinant Human TIMM8A His Protein

• DDP1deafness/dystonia peptide
• DDPMGC12262
• Deafness dystonia protein 1
• DFN1
• mitochondrial import inner membrane translocase subunit Tim8 A
• MTSTIM8
• TIM8A
• translocase of inner mitochondrial membrane 8 (yeast) homolog A
• translocase of inner mitochondrial membrane 8 homolog A (yeast)
• X-linked deafness dystonia protein

Background

TIMM8A 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. Recombinant human TIMM8A proten, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Peptides and proteins are guaranteed for 3 months from date of receipt.

Bioinformatics

• Gene Symbol: TIMM8A