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Anti-GCH1 Antibody (CAB10616)

SKU:
CAB10616
Product Type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism
€139 - €419
Frequently bought together:

Description

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Antibody Name:Anti-GCH1 Antibody
Antibody SKU:CAB10616
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human GCH1 (NP_000152.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:THP-1, Mouse liver, Mouse brain, Mouse spleen, Rat lung, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human GCH1 (NP_000152.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MEKG PVRA PAEK PRGA RCSN GFPE RDPP RPGP SRPA EKPP RPEA KSAQ PADG WKGE RPRS EEDN ELNL PNLA AAYS SILS SLGE NPQR QGLL KTPW RAAS AMQF FTKG YQET ISDV LNDA IFDE DHDE MVIV KDID MFSM CEHH LVPF VGKV HIGY LPNK QVLG LSKL ARIV EIYS RRLQ VQER LTKQ IAVA ITEA LRPA GVGV VVEA THMC MVMR GVQK MNSK TVTS TMLG VFRE DPKT REEF LTLI RS
Gene ID:2643
Uniprot:P30793
Cellular Location:Cytoplasm, Nucleus
Calculated MW:23kDa/25kDa/27kDa
Observed MW:28KDa
Synonyms:GCH1, DYT14, DYT5, DYT5a, GCH, GTP-CH-1, GTPCH1, HPABH4B
Background:This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7, 8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme.
NCBI Summary:This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme. [provided by RefSeq, Jul 2008]
UniProt Code:P30793
NCBI GenInfo Identifier:399536
NCBI Gene ID:2643
NCBI Accession:P30793.1
UniProt Related Accession:P30793
Molecular Weight:27kDa
NCBI Full Name:GTP cyclohydrolase 1
NCBI Synonym Full Names:GTP cyclohydrolase 1
NCBI Official Symbol:GCH1  
NCBI Official Synonym Symbols:GCH; DYT5; DYT14; DYT5a; GTPCH1; HPABH4B; GTP-CH-1  
NCBI Protein Information:GTP cyclohydrolase 1
UniProt Protein Name:GTP cyclohydrolase 1
UniProt Synonym Protein Names:GTP cyclohydrolase I
Protein Family:GTP cyclohydrolase
UniProt Gene Name:GCH1  
UniProt Entry Name:GCH1_HUMAN
Anti-GCH1 Antibody (CAB10616)Western blot analysis of extracts of various cell lines, using GCH1 antibody (CAB10616) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.

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