Cbs Antibody (PACO39514)

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SKU:
PACO39514
Product Type:
Antibody
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Applications:
ELISA
Applications:
IF
Antibody Type:
Polyclonal Antibody
Conjugation:
Unconjugated

Description

system_update_altDatasheetsystem_update_altMSDS
Antibody Name:Cbs Antibody (PACO39514)
Antibody SKU:PACO39514
Size:50ug
Host Species:Rabbit
Tested Applications:ELISA, IF
Recommended Dilutions:ELISA:1:2000-1:10000, IF:1:50-1:200
Species Reactivity:Mouse
Immunogen:Recombinant Mouse Cystathionine beta-synthase protein (2-561AA)
Form:Liquid
Storage Buffer:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Purification Method:>95%, Protein G purified
Clonality:Polyclonal
Isotype:IgG
Conjugate:Non-conjugated
Product ImageImmunofluorescent analysis of Hela cells using PACO39514 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Background:Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine. Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons.
Synonyms:Cystathionine beta-synthase (EC 4.2.1.22) (Beta-thionase) (Serine sulfhydrase), Cbs
UniProt Protein Function:CBS: Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury. Defects in CBS are the cause of cystathionine beta- synthase deficiency (CBSD). CBSD is an enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine. Belongs to the cysteine synthase/cystathionine beta- synthase family. 2 isoforms of the human protein are produced by alternative splicing.Protein type: EC 4.2.1.22; Amino Acid Metabolism - glycine, serine and threonine; Other Amino Acids Metabolism - selenoamino acid; Amino Acid Metabolism - cysteine and methionine; LyaseCellular Component: cytoplasm; cytosol; nucleusMolecular Function: cystathionine beta-synthase activity; cysteine synthase activity; enzyme binding; heme binding; identical protein binding; lyase activity; metal ion binding; nitrite reductase (NO-forming) activity; oxygen binding; protein homodimerization activity; pyridoxal phosphate binding; ubiquitin protein ligase bindingBiological Process: amino acid biosynthetic process; blood vessel remodeling; cerebellum morphogenesis; cysteine biosynthetic process; cysteine biosynthetic process from serine; cysteine biosynthetic process via cystathionine; endochondral ossification; homocysteine metabolic process; maternal process involved in pregnancy; negative regulation of apoptosis; regulation of blood vessel size; regulation of cGMP metabolic process; regulation of JNK activity; response to folic acid; superoxide metabolic process; transsulfuration
UniProt Protein Details:
NCBI Summary:
UniProt Code:Q91WT9
NCBI GenInfo Identifier:405778333
NCBI Gene ID:12411
NCBI Accession:NP_001258282.1
UniProt Secondary Accession:Q91WT9,Q91WU3
UniProt Related Accession:Q91WT9
Molecular Weight:63.4 kDa
NCBI Full Name:cystathionine beta-synthase isoform 2
NCBI Synonym Full Names:cystathionine beta-synthase
NCBI Official Symbol:Cbs  
NCBI Official Synonym Symbols:HIP4; AI047524; AI303044  
NCBI Protein Information:cystathionine beta-synthase
UniProt Protein Name:Cystathionine beta-synthase
UniProt Synonym Protein Names:Beta-thionase; Serine sulfhydrase
Protein Family:Cystathionine beta-synthase
UniProt Gene Name:Cbs  
UniProt Entry Name:CBS_MOUSE
ELISA Kits
Mouse Cystathionine beta-synthase (Cbs) ELISA Kit
Secondary Antibody
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)
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