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  4. Recombinant Human Glutaryl-CoA dehydrogenase, mitochondrial (GCDH)

Recombinant Human Glutaryl-CoA dehydrogenase, mitochondrial (GCDH)

  • 中文名稱：
    人GCDH重組蛋白
  • 貨號：
    CSB-YP849798HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人GCDH重組蛋白
  • 貨號：
    CSB-EP849798HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人GCDH重組蛋白
  • 貨號：
    CSB-EP849798HU-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    人GCDH重組蛋白
  • 貨號：
    CSB-BP849798HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人GCDH重組蛋白
  • 貨號：
    CSB-MP849798HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    GCDH
  • Uniprot No.：
  • 別名：
    ACAD5 ; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase; Glutaryl CoA dehydrogenase; mitochondrial ; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    45-438
  • 氨基酸序列
    RPEFDW QDPLVLEEQL TTDEILIRDT FRTYCQERLM PRILLANRNE VFHREIISEM GELGVLGPTI KGYGCAGVSS VAYGLLAREL ERVDSGYRSA MSVQSSLVMH PIYAYGSEEQ RQKYLPQLAK GELLGCFGLT EPNSGSDPSS METRAHYNSS NKSYTLNGTK TWITNSPMAD LFVVWARCED GCIRGFLLEK GMRGLSAPRI QGKFSLRASA TGMIIMDGVE VPEENVLPGA SSLGGPFGCL NNARYGIAWG VLGASEFCLH TARQYALDRM QFGVPLARNQ LIQKKLADML TEITLGLHAC LQLGRLKDQD KAAPEMVSLL KRNNCGKALD IARQARDMLG GNGISDEYHV IRHAMNLEAV NTYEGTHDIH ALILGRAITG IQAFTASK
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產品評價

靶點詳情

  • 功能：
    Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
  • 基因功能參考文獻：
    1. Molecular genetics analysis identified 14 different mutations in the GCDH gene in the 18 patients with Glutaric acidemia I PMID: 28389991
    2. Four mutations of the glutaryl-CoA dehydrogenase (GCDH) gene were identified among the patients with diagnosis of glutaric acidemia type I (GA-I). PMID: 29419857
    3. We report the allele frequencies for three known Glutaric aciduria type I low excretors GCDH variants (M405V, V400M and R227P) and note that both the M405V and V400M variants are significantly more common in the population of African ancestry compared to the general population PMID: 27397597
    4. Our data underscore the impact of GCDH protein interactions mediated by amino acid residues on the surface of GCDH required for proper enzymatic activity PMID: 28062662
    5. Mutations in GCDH gene observed in the present study indicate genetic heterogeneity in GCDH gene among South Indian population. No definite genotype-phenotype correlations were observed. PMID: 26071121
    6. 2 novel mutations, p.Glu64Asp and p.Gly268Val, account for majority of disease alleles in Cypriot patients with Glutaric aciduria type I; a founder effect for the p.Glu64Asp and the p.Gly268Val can be suggested based on place of origin of mutation carriers PMID: 24973495
    7. Point mutation of GCDH gene is associated with glutaric academia type I. PMID: 25863083
    8. 29 GCDH mutations were identified in 23 glutaric aciduria type 1 patients, including 11 novel mutations PMID: 24332224
    9. Data indicate a homozygous c.1244-2A> C mutation of the glutaryl-CoA dehydrogenase (GCDH) gene in both patients. PMID: 25297592
    10. These cells displayed decreased levels of GCDH tetramer. PMID: 22231382
    11. Identification of GCDH gene mutations in four patients with glutaric academia type I. PMID: 23225040
    12. A homozygous, disease-segregating mutation (p.Val400Met) was identified in the glutaryl-CoA dehydrogenase (GCDH) gene at chromosome 19p13. PMID: 21912879
    13. physiological concentrations of flavin adenine dinucleotide resulted in a spectacular enhancement of the thermal stabilities of GCDH and prevented enzymatic activity loss PMID: 21968293
    14. GCDH gene mutations are identified in 8 patients with glutaric aciduria type I PMID: 21811973
    15. 12 glutaric aciduria type 1 patients were found homozygous for the same A293T mutation in the glutaryl-CoA dehydrogenase (GCDH) gene. PMID: 20732827
    16. mutational analysis of glutaryl-CoA dehydrogenase in two patients with glutaric aciduria type 1. PMID: 20514322
    17. Cerebral toxicity caused by GCDH deficiency may induce a state of arteriolar dilation and increased cerebral blood volume. PMID: 20032085
    18. Three-dimensional structures of human GCD in uncomplexed form and in complex with 4-nitrobutyryl-CoA are reported, and the structural bases for the mechanisms of the dehydrogenation and decarboxylation reactions are proposed. PMID: 15274622
    19. The authors report two GCDH-deficient patients with macrocephaly presenting with progressive neurologic deterioration and a severe leukoencephalopathy during adolescence or adulthood. PMID: 15985591
    20. An autosomal recessive disease thsat leads to an accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitine deficiency, (review) PMID: 16368216
    21. The major rate-determining step in the steady-state turnover of glutaryl-CoA dehydrogenase (GCD) occurs at the release of crotonyl-CoA product; the chemical steps and reoxidation of reduced FAD are much faster than the turnover of wild-type GCD. PMID: 17176108
    22. GA I is caused by mutations in the GCDH gene, encoding glutaryl-CoA dehydrogenase. PMID: 17661081
    23. Expression studies of four missense mutations in GCDH indicate that both enzyme instability and impaired enzyme function can underlie the autosomal recessive neurometabolic disorder glutaric aciduria type I. PMID: 18775954
    24. In the oxidative decarboxylation of glutaryl-coenzyme A (CoA) that is catalyzed by glutaryl-CoA dehydrogenase, glutaconyl-CoA is the presumed enzyme-bound intermediate. PMID: 11705404

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  • 相關疾病：
    Glutaric aciduria 1 (GA1)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Acyl-CoA dehydrogenase family
  • 組織特異性：
    Isoform Long and isoform Short are expressed in fibroblasts and liver.
  • 數據庫鏈接：

    HGNC: 4189

    OMIM: 231670

    KEGG: hsa:2639

    STRING: 9606.ENSP00000222214

    UniGene: Hs.532699



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