BCKDHB (NM_183050) Human Mass Spec Standard

CAT#: PH308059

BCKDHB MS Standard C13 and N15-labeled recombinant protein (NP_898871)

Product Images

Coomassie blue staining of purified BCKDHB protein (Cat# TP308059). The protein was produced from HEK293T cells transfected with BCKDHB cDNA clone (Cat# RC208059) using MegaTran 2.0 (Cat# TT210002).

Specifications

Product Data

• Tag: C-Myc/DDK
• Species: Human
• Expression Host: HEK293
• Expression cDNA Clone or AA Sequence: RC208059
• Predicted MW: 43.1 kDa
• Protein Sequence:
  Sequence Link

  >RC208059 protein sequence
  Red=Cloning site Green=Tags(s)
  
  MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDPEPREYGQTQK
  MNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTG
  ATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGI
  KVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQ
  VHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECF
  LNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
  
  TRTRPLEQKLISEEDLAANDILDYKDDDDKV
• Purity: > 80% as determined by SDS-PAGE and Coomassie blue staining
• Concentration: 50 ug/ml as determined by BCA
• Labeling Method: Labeled with [U- 13C6, 15N4]-L-Arginine and [U- 13C6, 15N2]-L-Lysine
• Buffer: 100 mM glycine, 25 mM Tris-HCl, pH 7.3. Store at -80°C. Avoid repeated freeze-thaw cycles. Stable for 3 months from receipt of products under proper storage and handling conditions.

Reference Data

• RefSeq: NP_898871
• RefSeq Size: 3712
• RefSeq ORF: 1176
• Synonyms: BCKDE1B; BCKDH E1-beta; E1B
• Locus ID: 594
• UniProt ID: P21953, A0A140VKB3
• Cytogenetics: 6q14.1
• Summary: 'This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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 multiple transcript variants. [provided by RefSeq, Jan 2016]'
• Protein Pathways: Metabolic pathways, Valine, leucine and isoleucine degradation