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AV38672

Sigma-Aldrich

Anti-TSFM (AB2) antibody produced in rabbit

IgG fraction of antiserum

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Synonym(s):
Anti-Ts translation elongation factor, mitochondrial
NACRES:
NA.41

biological source

rabbit

Quality Level

conjugate

unconjugated

antibody form

IgG fraction of antiserum

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

38 kDa

species reactivity

human, rabbit, horse, bovine, guinea pig, dog

concentration

0.5 mg - 1 mg/mL

technique(s)

western blot: suitable

NCBI accession no.

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... TSFM(10102)

Immunogen

Synthetic peptide directed towards the C terminal region of human TSFM

Biochem/physiol Actions

Ts translation elongation factor, mitochondrial (TSFM) is a guanine nucleotide exchange factor for EFTu during the elongation step of protein translation in mitochondria. Mutations in TSFM gene results in combined oxidative phosphorylation deficiency-3 syndrome.

Sequence

Synthetic peptide located within the following region: VVGMAPLSVGSLDDEPGGEAETKMLSQPYLLDPSITLGQYVQPQGVSVVD

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Information

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Jan A M Smeitink et al.
American journal of human genetics, 79(5), 869-877 (2006-10-13)
The 13 polypeptides encoded in mitochondrial DNA (mtDNA) are synthesized in the mitochondrial matrix on a dedicated protein-translation apparatus that resembles that found in prokaryotes. Here, we have investigated the genetic basis for a mitochondrial protein-synthesis defect associated with a
Assignment of the mitochondrial translation elongation factor Ts gene (TSFM) to human chromosome 12 bands q13-->q14 by in situ hybridization and with somatic cell hybrids.
J L Vernon et al.
Cytogenetics and cell genetics, 89(3-4), 145-146 (2000-08-31)
Sonia Emperador et al.
European journal of human genetics : EJHG, 25(1), 153-156 (2016-09-30)
Oxidative phosphorylation dysfunction has been found in many different disorders. This biochemical pathway depends on mitochondrial protein synthesis. Thus, mutations in components of the mitochondrial translation system can be responsible for some of these pathologies. We identified a new homozygous

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