TIMM8A Antibody
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.
NCBI Gene ID
1678
Swiss Prot
O60220
Host
Rabbit
Reactivity
Human, Mouse
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 1-97 of human TIMM8A (NP_004076.1) .
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Neuroscience, Signal Transduction
Purification
Affinity purification
Positive Control
U-251MG
Concentration
Batch dependent
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
Store at -20˚ C. Avoid freeze / thaw cycles.
Product Datasheet
https://www.prosci-inc.com/?datasheet_sku=23-916
Calculated Molecular Weight
Observed: 11kDa
Fragment
IgG
Applications Notes
WB: 1:500 - 1:2000
Symbol
TIMM8A
Positive Control 2
A-549
Positive Control 3
LO2
Positive Control 4
293T
Positive Control 5
MCF7
Positive Control 6
HeLa
NCBI Official Name
Translocase of inner mitochondrial membrane 8A
NCBI Organism
Homo sapiens
Other Product Names
DDP, DDP1, DFN1, MTS, TIM8, mitochondrial import inner membrane translocase subunit Tim8 A, X-linked deafness dystonia protein, deafness dystonia protein 1, deafness/dystonia peptide, translocase of inner mitochondrial membrane 8 homolog A
Tested Applications
WB
Physical Properties
Liquid
Curated Selection
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