BCKDHB anticorps (N-Term)

N° du produit ABIN2787755

Cet anticorps Lapin Polyclonal détecte spécifiquement BCKDHB dans WB. Il présente une réactivité avec des échantillons de Humain, Rat, Boeuf (Vache), Chien, Cheval, Porc et Lapin.

Aperçu rapide pour BCKDHB anticorps (N-Term) (ABIN2787755)

Antigène: BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))

Reactivité: Humain, Rat, Boeuf (Vache), Chien, Cheval, Porc, Lapin

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp BCKDHB est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-BCKDHB anticorps

Épitope: N-Term

Fonction: BCKDHB Antibody - N-terminal region

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

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Commentaires:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP58593-100UG

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: 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: prevent freeze-thaw cycles

Stock: 4 °C,-20 °C

Stockage commentaire: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Détails sur BCKDHB

Antigène: BCKDHB (Branched Chain Keto Acid Dehydrogenase E1, beta Polypeptide (BCKDHB))

Autre désignation: BCKDHB

Sujet:

Background Information: 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.

Gene Name: branched chain keto acid dehydrogenase E1, beta polypeptide

Alternative Symbols: E1B, BCKDE1B, BCKDH E1-beta

Protein Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial Ensembl ENSP00000443564 Ensembl ENSP00000358775

Poids moléculaire: 23kDa

ID gène: 594

UniProt: Q5T2J3