Anti-TUFM Antibody Picoband® 31.0647 Vervoeging

Elongation factor Tu, mitochondrial is a protein that in humans is encoded by the TUFM gene. It is an EF-Tu homolog. This gene encodes a protein which participates in protein translation in mitochondria. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency resulting in lactic acidosis and fatal encephalopathy. A pseudogene has been identified on chromosome 17.

Phosphopantothenate--cysteine ligase, Phosphopantothenoylcysteine synthetase, PPC synthetase, PPCS, COAB

TUFM

7284

P49411

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human TUFM recombinant protein (Position: R8-R271) .

Expressed in heart, placenta, skeletal muscle kidney, pancreas, spleen, prostate, testis, ovary, ileum and colon. Expressed in lung endothelial and smooth muscle cells (at protein level) .

Flow Cytometry

Amino Acid Metabolism, Amino Acids, Cofactors, Vitamins/Minerals, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Signal Transduction, Vitamins/Minerals

Immunogen affinity purified.

Liquid

Catalyzes the first step in the biosynthesis of coenzyme A from vitamin B5, where cysteine is conjugated to 4'-phosphopantothenate to form 4-phosphopantothenoylcysteine.

1. Bogenhagen, D. F., Rousseau, D., Burke, S. The layered structure of human mitochondrial DNA nucleoids. J. Biol. Chem. 283: 3665-3675, 2008. 2. Kohda, M., Tokuzawa, Y., Kishita, Y., Nyuzuki, H., Moriyama, Y., Mizuno, Y., Hirata, T., Yatsuka, Y., Yamashita-Sugahara, Y., Nakachi, Y., Kato, H., Okuda, A., and 23 others. A comprehensive genomic analysis reveals the genetic landscape of mitochondrial respiratory chain complex deficiencies. PLoS Genet. 12: e1005679, 2016. Note: Electronic Article. 3. Liljas, A., Ehrenberg, M., Aqvist, J. Comment on 'The mechanism for activation of GTP hydrolysis on the ribosome.' Science 333: 37 only, 2011.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

48608 MW

6

Phosphopantothenoylcysteine synthetase

Cytosol.

Serine protease HTRA3

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.