Recombinant Human Cystathionine beta-synthase | MyBioSource MBS968346 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Cystathionine beta-synthase

catalog :

MBS968346

quantity :

0.05 mg (E-Coli)

price :

180 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS968346

products type :

Recombinant Protein

products full name :

Recombinant Human Cystathionine beta-synthase

products short name :

Cystathionine beta-synthase

products name syn :

Beta-thionase; Serine sulfhydrase

other names :

cystathionine beta-synthase; Cystathionine beta-synthase; cystathionine beta-synthase; cystathionine-beta-synthase; Beta-thionase; Serine sulfhydrase

products gene name :

CBS

other gene names :

CBS; CBS; HIP4

uniprot entry name :

CBS_HUMAN

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

2-551; Full length

sequence length :

551

sequence :

AAQERDQK
PSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKE
PLWIRPDAPSRCTWQLGRPASESPHHHTAPAKSPKILPD
ILKKIGDTPMVRINKIGKKFGLKCELLAKCEFFNAGGSV
KDRISLRMIEDAERDGTLKPGDTIIEPTSGNTGIGLALA
AAVRGYRCIIVMPEKMSSEKVDVLRALGAEIVRTPTNAR
FDSPESHVGVAWRLKNEIPNSHILDQYRNASNPLAHYDT
TADEILQQCDGKLDMLVASVGTGGTITGIARKLKEKCPG
CRIIGVDPEGSILAEPEELNQTEQTTYEVEGIGYDFIPT
VLDRTVVDKWFKSNDEEAFTFARMLIAQEGLLCGGSAGS
TVAVAVKAAQELQEGQRCVVILPDSVRNYMTKFLSDRWM
LQKGFLKEEDLTEKKPWWWHLRVQELGLSAPLTVLPTIT
CGHTIEILREKGFDQAPVVDEAGVILGMVTLGNMLSSLL
AGKVQPSDQVGKVIYKQFKQIRLTDTLGRLSHILEMDHF
ALVVHEQIQYHSTGKSSQRQMVFGVVTAIDLLNFV

purity :

Greater than 90% as determined by SDS-PAGE.

form :

Liquid containing glycerol; lyophilization may be available upon request.

storage stability :

Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

products categories :

Metabolism

products description :

Only known pyridoxal phosphate-dependent enzyme that contains he. 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.

products references :

Human cystathionine beta-synthase cDNA sequence, alternative splicing and expression in cultured cells.Kraus J.P., Le K., Swaroop M., Ohura T., Tahara T., Rosenberg L.E., Roper M.D., Kozich V.Hum. Mol. Genet. 2:1633-1638(1993)

ncbi gi num :

4557415

ncbi acc num :

NP_000062.1

ncbi gb acc num :

NM_000071.2

uniprot acc num :

P35520

ncbi mol weight :

64.5kD

ncbi pathways :

Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Cysteine And Methionine Metabolism Pathway (104488); Cysteine And Methionine Metabolism Pathway (103421); Cysteine Biosynthesis, Homocysteine + Serine = Cysteine Pathway (413414); Cysteine Biosynthesis, Homocysteine + Serine = Cysteine Pathway (468303); Cysteine Formation From Homocysteine Pathway (1270182); Folate-Alcohol And Cancer Pathway (920980); Glycine, Serine And Threonine Metabolism Pathway (82949); Glycine, Serine And Threonine Metabolism Pathway (313)

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 summary :

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: Amino Acid Metabolism - cysteine and methionine; Lyase; Amino Acid Metabolism - glycine, serine and threonine; EC 4.2.1.22; Other Amino Acids Metabolism - selenoamino acid. Chromosomal Location of Human Ortholog: 21q22.3. Cellular Component: cytoplasm; cytosol; nucleus. Molecular Function: cystathionine beta-synthase activity; cysteine synthase activity; enzyme binding; heme binding; identical protein binding; metal ion binding; nitrite reductase (NO-forming) activity; oxygen binding; protein binding; protein homodimerization activity; pyridoxal phosphate binding; ubiquitin protein ligase binding. Biological Process: cysteine biosynthetic process; cysteine biosynthetic process from serine; cysteine biosynthetic process via cystathionine; DNA protection; homocysteine catabolic process; homocysteine metabolic process; L-cysteine catabolic process; L-serine catabolic process; L-serine metabolic process; selenium metabolic process; sulfur amino acid metabolic process; transsulfuration. Disease: Homocystinuria Due To Cystathionine Beta-synthase Deficiency

size1 :

0.05 mg (E-Coli)

price1 :

180 USD

size2 :

0.2 mg (E-Coli)

price2 :

490

size3 :

0.05 mg (Baculovirus)

price3 :

715

size4 :

0.5 mg (E-Coli)

price4 :

715

size5 :

1 mg (E-Coli)

price5 :

1170

more info or order :

MyBioSource product webpage