Anti-CBS Antibody (CAB11612)

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SKU:
CAB11612
Product type:
Antibody
Reactivity:
Human
Mouse
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Monoclonal Antibody
Research Area:
Metabolism
Frequently bought together:

Description

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Antibody Name:Anti-CBS Antibody
Antibody SKU:CAB11612
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human CBS
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse, Rat
Positive Samples:HepG2, MCF7, Mouse liver, Mouse kidney, Rat liver
Immunogen:A synthesized peptide derived from human CBS
Purification Method:Affinity purification
Storage Buffer:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:875
Uniprot:P35520
Cellular Location:
Calculated MW:61kDa
Observed MW:61KDa
Anti-CBS AntibodyWestern blot - CBS Rabbit mAb (CAB11612)
Synonyms:HIP4
Background:The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016]
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.
UniProt Protein Details:

Protein type:Other Amino Acids Metabolism - selenoamino acid; EC 4.2.1.22; Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - cysteine and methionine; Lyase

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: intracellular membrane-bound organelle; nucleolus; cytosol; nucleus

Molecular Function:identical protein binding; protein binding; enzyme binding; protein homodimerization activity; cystathionine beta-synthase activity; ubiquitin protein ligase binding; metal ion binding; heme binding; pyridoxal phosphate binding

Biological Process: cysteine biosynthetic process via cystathionine; homocysteine catabolic process; L-serine metabolic process; sulfur amino acid metabolic process; transsulfuration; cysteine biosynthetic process from serine; homocysteine metabolic process; L-serine catabolic process; L-cysteine catabolic process

Disease: Homocystinuria Due To Cystathionine Beta-synthase Deficiency

NCBI Summary:The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, May 2010]
UniProt Code:P35520
NCBI GenInfo Identifier:543959
NCBI Gene ID:875
NCBI Accession:P35520.2
UniProt Secondary Accession:P35520,Q99425, Q9BWC5, B2R993, D3DSK4,
UniProt Related Accession:P35520
Molecular Weight:61,863 Da
NCBI Full Name:Cystathionine beta-synthase
NCBI Synonym Full Names:cystathionine-beta-synthase
NCBI Official Symbol:CBS  
NCBI Official Synonym Symbols:HIP4  
NCBI Protein Information:cystathionine beta-synthase; beta-thionase; serine sulfhydrase; methylcysteine 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_HUMAN

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