HADHA Rabbit Polyclonal Antibody

CAT#: TA318956

Rabbit polyclonal anti-TFP1/HADHA antibody

Product Images

Western blot analysis of TFP-1 expression with lysate from Jurkat cells (Lane 1, 2), 3T3 cells (Lane 3) and rat kidney (Lane 4).

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB: 0.5-4 ug/ml
• Reactivities: Human, Mouse, Rat
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Synthetic peptide surrounding amino acid 750 of human TFP1
• Formulation: 100 µg (0.5 mg/ml) affinity purified rabbit polyclonal antibody in 1X phosphate-buffered saline (PBS) containing 30% glycerol, 0.5% BSA, and 0.01% thimerosal.
• Concentration: lot specific
• Purification: Affinity purified
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Gene Name: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
• Database Link:
  NP_000173
  Entrez Gene 97212 MouseEntrez Gene 170670 RatEntrez Gene 3030 Human
  UniProt ID P40939
• Background: Mitochondrial Trifunctional Protein (TFP) is a multienzyme complex of the beta-oxidation cycle. TFP deficiency is a clinically heterogeneous disorder with phenotypes of different severity. The spectrum of diseases range from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. Human TFP is an octomer composed of four alpha-subunits and four beta-subunits. Mutations in either subunits may result in general TFP deficiency with reduced activity of all enzymes.
• Synonyms: ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA

Reference Data

• Protein Families: Druggable Genome
• Protein Pathways: beta-Alanine metabolism, Biosynthesis of unsaturated fatty acids, Butanoate metabolism, Fatty acid elongation in mitochondria, Fatty acid metabolism, Limonene and pinene degradation, Lysine degradation, Metabolic pathways, Propanoate metabolism, Tryptophan metabolism, Valine, leucine and isoleucine degradation