PCCA Antibody
-
中文名稱：PCCA兔多克隆抗體
-
貨號：CSB-PA017522LA01HU
-
規(guī)格：￥440
-
圖片：
-
Western Blot
Positive WB detected in: NIH/3T3 whole cell lysate, Hela whole cell lysate
All lanes: PCCA antibody at 4µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 81, 78, 76 kDa
Observed band size: 81 kDa -
Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA017522LA01HU at dilution of 1:100 -
Immunohistochemistry of paraffin-embedded human heart tissue using CSB-PA017522LA01HU at dilution of 1:100
-
-
其他：
產品詳情
-
產品名稱：Rabbit anti-Homo sapiens (Human) PCCA Polyclonal antibody
-
Uniprot No.：
-
基因名：
-
別名：mitochondrial antibody; Pcca antibody; pccA COMPLEMENTATION GROUP antibody; PCCA_HUMAN antibody; PCCase subunit alpha antibody; Propanoyl CoA:carbon dioxide ligase subunit alpha antibody; Propanoyl-CoA:carbon dioxide ligase subunit alpha antibody; Propionyl CoA carboxylase alpha chain antibody; Propionyl CoA carboxylase alpha chain mitochondrial antibody; Propionyl CoA carboxylase alpha polypeptide antibody; Propionyl coenzyme A carboxylase alpha polypeptide antibody; Propionyl-CoA carboxylase alpha chain antibody
-
宿主：Rabbit
-
反應種屬：Human, Mouse
-
免疫原：Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial protein (585-720AA)
-
免疫原種屬：Homo sapiens (Human)
-
標記方式：Non-conjugated
本頁面中的產品，PCCA Antibody (CSB-PA017522LA01HU)，的標記方式是Non-conjugated。對于PCCA Antibody，我們還提供其他標記。見下表：
-
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, WB, IHC
-
推薦稀釋比：
Application Recommended Dilution WB 1:500-1:5000 IHC 1:20-1:200 -
Protocols：
-
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
相關產品
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：
- 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). The SNP c.937C>T (p.Arg313Ter), was noted in 9/36 (25%) alleles in the PCCA gene PMID: 27227689
- Ten propionic acidemia mutations were confirmed, including 8 affecting the PCCA gene and 2 affecting the PCCB gene PMID: 25636094
- Two PCCA mutations, c.229C-->T (p.R77W) and c.1262A-->C (p.Q421P), were identified in a PCCA-deficient patient. PMID: 24863100
- Mutation analysis of the PCCA gene identified homozygous c.1284+1G>A in patient 1, c.230G>A (p.R77Q) and c.1855C>T (p.R619X) in patient 2, homozygous c.2125T>C (p.S709P) in patient 3, and only one mutant allele, c.231+1G>T in patient 4. PMID: 24464666
- Thsi study presented that Propionic acidemia(PCCA mutation) associated with visual hallucinations PMID: 22156789
- propionyl CoA carboxylase alpha polypeptide intronic variations causing aberrantly spliced messenger RNA is associated with propionic and methylmalonic acidemia. PMID: 17966092
- cryo-electron microscopy (cryo-EM) reconstruction at 15-A resolution PMID: 20725044
- pathogenicity of 11 mutations by expression studies and correlation of genotype-phenotype in PCCA-deficient propionic acidemia patients. PMID: 12385775
- Data reported 9 novel PCCA gene mutations and represents an extensive update of the mutational study of propionic acidemia providing important information about the worldwide distribution of PA mutations. PMID: 12559849
- analysis of propionyl-CoA carboxylase containing pathogenic mutations in the beta subunit (R165W, E168K, and R410W) and one PCCB polymorphism (A497V) and their structural and functional effects PMID: 15890657
- analysis of PCCA and PCCB mutations in propionic acidemia PMID: 17051315
- This work describes for the first time the high frequency of large genomic deletions in the PCCA gene, which could be due to the characteristics of the PCCA gene structure and its abundance in intronic repetitive elements. PMID: 19157943
- Activities of propionyl-CoA carboxylase were not significantly different in pancreatic islets of patients with type 2 diabetes from those of the control. PMID: 19296078
顯示更多
收起更多
-
相關疾?。?/div>Propionic acidemia type I (PA-1)亞細胞定位：Mitochondrion matrix.數據庫鏈接：
Most popular with customers
-
-
YWHAB Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC, IF, FC
Species Reactivity: Human, Mouse, Rat
-
Phospho-YAP1 (S127) Recombinant Monoclonal Antibody
Applications: ELISA, WB, IHC
Species Reactivity: Human
-
-
-
-
-
