Human TSFM Recombinant Protein (RPPB5028)

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SKU:
RPPB5028
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
TSFM
Synonyms:
Elongation factor Ts
Synonyms:
mitochondrial
Synonyms:
Ts Translation Elongation Factor Mitochondrial
Synonyms:
TSFM
Source:
Escherichia Coli
Uniprot:
P43897
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Description

Product Name:Human TSFM Recombinant Protein
Product Code:RPPB5028
Size:20µg
Species:Human
Target:TSFM
Synonyms:Elongation factor Ts, mitochondrial, Ts Translation Elongation Factor Mitochondrial, TSFM, EF-Ts, EF-TsMt, COXPD3.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:TSFM protein solution (1mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 10% glycerol and 0.4M Urea.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 85.0% as determined by SDS-PAGE.
Amino Acid Sequence:MSKELLMKLR RKTGYSFVNC KKALETCGGD LKQAEIWLHK EAQKEGWSKA AKLQGRKTKE GLIGLLQEGN TTVLVEVNCE TDFVSRNLKF QLLVQQVALG TMMHCQTLKD QPSAYSKVQW LTPVNLALWE AEAGGSLEGF LNSSELSGLP AGPDREGSLK DQLALAIGKL GENMILKRAA WVKVPSGFYV GSYVHGAMQS PSLHKLVLGK YGALVICETS EQKTNLEDVG RRLGQHVVGM APLSVGSLDD EPGGEAETKM LSQPYLLDPS ITLGQYVQPQ GVSVVDFVRF ECGEGEEAAE TE

TSFM is a mitochondrial translation elongation factor which is linked with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. TSFM stays bound to the aminoacyl-tRNA.EF-Tu.GTP complex until the GTP hydrolysis stage on the ribosome. Mutations in TSFM are related with combined oxidative phosphorylation deficiency-3 syndrome.

TSFM Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 302 amino acids (46-346 a.a) and having a molecular mass of 32.9kDa.TSFM is purified by proprietary chromatographic techniques.

UniProt Protein Function:EFT5: Associates with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. It remains bound to the aminoacyl-tRNA.EF- Tu.GTP complex up to the GTP hydrolysis stage on the ribosome. Defects in TSFM are the cause of combined oxidative phosphorylation deficiency type 3 (COXPD3). Defects in the mitochondrial oxidative phosphorylation system result in devastating, mainly multisystem, diseases. COXPD3 symptoms include severe metabolic acidosis with encephalomyopathy or with hypertrophic cardiomyopathy. Patients show a severe defect in mitochondrial translation leading to a failure to assemble adequate amounts of three of the oxidative phosphorylation complexes. Belongs to the EF-Ts family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Translation elongation; Translation

Chromosomal Location of Human Ortholog: 12q14.1

Cellular Component: mitochondrial matrix; mitochondrion; nucleus

Molecular Function:translation elongation factor activity

Biological Process: regulation of RNA elongation; translational elongation

Disease: Combined Oxidative Phosphorylation Deficiency 3

NCBI Summary:This gene encodes a mitochondrial translation elongation factor. The encoded protein is an enzyme that catalyzes the exchange of guanine nucleotides on the translation elongation factor Tu during the elongation step of mitchondrial protein translation. Mutations in this gene are associated with combined oxidative phosphorylation deficiency-3 syndrome. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Mar 2010]
UniProt Code:P43897
NCBI GenInfo Identifier:12644268
NCBI Gene ID:10102
NCBI Accession:P43897.2
UniProt Secondary Accession:P43897,Q561V7, Q8TBC2, Q9UQK0, B4E391, F5H2T7,
UniProt Related Accession:P43897
Molecular Weight:23,557 Da
NCBI Full Name:Elongation factor Ts, mitochondrial
NCBI Synonym Full Names:Ts translation elongation factor, mitochondrial
NCBI Official Symbol:TSFM  
NCBI Official Synonym Symbols:EFTS; EFTSMT  
NCBI Protein Information:elongation factor Ts, mitochondrial
UniProt Protein Name:Elongation factor Ts, mitochondrial
UniProt Gene Name:TSFM  
UniProt Entry Name:EFTS_HUMAN
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