PCCB Antibody, Biotin conjugated

說明書

中文名稱：

PCCB兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA017523LD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) PCCB Polyclonal antibody
Uniprot No.：

P05166
基因名：

PCCB
別名：

DKFZp451E113 antibody; PCCase subunit beta antibody; pccB antibody; PCCB_HUMAN antibody; pccBC Complementation group antibody; Propanoyl CoA:carbon dioxide ligase subunit beta antibody; Propanoyl-CoA:carbon dioxide ligase subunit beta antibody; Propionyl CoA carboxylase beta chain; mitochondrial antibody; propionyl Coenzyme A carboxylase; beta polypeptide antibody; Propionyl-CoA carboxylase ; beta subunit antibody; Propionyl-CoA carboxylase beta chain; mitochondrial antibody; R74805 antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial protein (200-314AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
基因功能參考文獻：
1. Recurrent lactic acid elevations were present in six out of the eight patients. Five of the eight patients were diagnosed with Autism Spectrum Disorder, four of them had pathogenic variants in PCCB PMID: 27825584
2. The majority of patients had mutations in the PCCA gene (18/25). A total of 26 mutations were noted: 20 in the PCCA gene and 6 in PCCB gene. Seventeen mutations were novel (14 in PCCA and 3 in PCCB). PMID: 27227689
3. The c.-4156_183+3713del mutation is the first known large deletion that affects the PCCB gene functions. PMID: 24863100
4. mutations affect homomeric and heteromeric assembly of Propionyl-CoA Carboxylase PMID: 11749052
5. Structure-function analysis of a range of isolated PCCB subunit mutants has elucidated the complex relationship between genotype and phenotype in propionic acidemia. PMID: 12007220
6. Work showed 15 novel PCCB gene mutations and phenotype-genotype correlations for the prediction of the metabolic outcome and for the implementation of treatments tailored to each PA patient. PMID: 12559849
7. pathogenicity of R67S, R165Q and G112D mutations in PCCB gene in propionic acidemia PMID: 12757933
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相關疾病：

Propionic acidemia type II (PA-2)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

AccD/PCCB family
數據庫鏈接：

HGNC: 8654

OMIM: 232050

KEGG: hsa:5096

STRING: 9606.ENSP00000419027

UniGene: Hs.63788

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