BCKDHB anticorps (N-Term)
Aperçu rapide pour BCKDHB anticorps (N-Term) (ABIN2787755)
Antigène
Voir toutes BCKDHB AnticorpsReactivité
Hôte
Clonalité
Conjugué
Application
-
-
Épitope
- N-Term
-
Séquence
- GFLHPAATVE DAAQRRQVAH FTFQPDPEPR EYGQTQKMNL FQSVTSALDN
-
Homologie
- Cow: 93%, Dog: 93%, Horse: 93%, Human: 100%, Pig: 86%, Rabbit: 93%, Rat: 79%
-
Attributs du produit
- This is a rabbit polyclonal antibody against BCKDHB. It was validated on Western Blot.
-
Purification
- Affinity Purified
-
Immunogène
- The immunogen is a synthetic peptide directed towards the N-terminal region of Human BCKDHB
-
-
-
-
Restrictions
- For Research Use only
-
-
-
Format
- Liquid
-
Concentration
- 1 mg/mL
-
Buffer
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
-
Agent conservateur
- Sodium azide
-
Précaution d'utilisation
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
Conseil sur la manipulation
- Avoid repeat freeze-thaw cycles.
-
Stock
- -20 °C
-
Stockage commentaire
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
-
-
- BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))
-
Autre désignation
- BCKDHB
-
Sujet
-
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Protein Interaction Partner: CUL3, BCKDHA,
Protein Size: 218 -
Poids moléculaire
- 23 kDa
-
ID gène
- 594
-
UniProt
- Q5T2J3
Antigène
-