Recombinant Human Sulfite oxidase, mitochondrial | MyBioSource MBS954246 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human Sulfite oxidase, mitochondrial

catalog :

MBS954246

quantity :

0.05 mg (E-Coli)

price :

190 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS954246

products type :

Recombinant Protein

products full name :

Recombinant Human Sulfite oxidase, mitochondrial

products short name :

Sulfite oxidase

other names :

sulfite oxidase, mitochondrial; Sulfite oxidase, mitochondrial; sulfite oxidase, mitochondrial; sulfite oxidase

products gene name :

SUOX

other gene names :

SUOX; SUOX

uniprot entry name :

SUOX_HUMAN

host :

E Coli or Yeast or Baculovirus or Mammalian Cell

sequence positions :

80-545

sequence length :

545

sequence :

ESTHIYTKEEVSSHTSPETGIWVTLGSEVFDVTEFVDLH
PGGPSKLMLAAGGPLEPFWALYAVHNQSHVRELLAQYKI
GELNPEDKVAPTVETSDPYADDPVRHPALKVNSQRPFNA
EPPPELLTENYITPNPIFFTRNHLPVPNLDPDTYRLHVV
GAPGGQSLSLSLDDLHNFPRYEITVTLQCAGNRRSEMTQ
VKEVKGLEWRTGAISTARWAGARLCDVLAQAGHQLCETE
AHVCFEGLDSDPTGTAYGASI

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 references :

Molecular cloning of human liver sulfite oxidase.Garrett R.M., Bellissimo D.B., Rajagopalan K.V.Biochim. Biophys. Acta 1262:147-149(1995) Genomic DNA sequence of human sulfite oxidase SUOX.Coyne K.E., Johnson J.L., Rajagopalan K.V.An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.J. Proteomics 96:253-262(2014) The 1.2 A structure of the human sulfite oxidase cytochrome b(5) domain.Rudolph M.J., Johnson J.L., Rajagopalan K.V., Kisker C.Acta Crystallogr. D 59:1183-1191(2003) Molecular basis of sulfite oxidase deficiency from the structure of sulfite oxidase.Kisker C., Schindelin H., Pacheco A., Wehbi W.A., Garrett R.M., Rajagopalan K.V., Enemark J.H., Rees D.C.Cell 91:973-983(1997) Human sulfite oxidase R160Q identification of the mutation in a sulfite oxidase-deficient patient and expression and characterization of the mutant enzyme.Garrett R.M., Johnson J.L., Graf T.N., Feigenbaum A., Rajagopalan K.V.Proc. Natl. Acad. Sci. U.S.A. 95:6394-6398(1998) Isolated sulfite oxidase deficiency review of two cases in one family.Edwards M.C., Johnson J.L., Marriage B., Graf T.N., Coyne K.E., Rajagopalan K.V., MacDonald I.M.Ophthalmology 106:1957-1961(1999) Isolated sulfite oxidase deficiency identification of 12 novel SUOX mutations in 10 patients.Johnson J.L., Coyne K.E., Garrett R.M., Zabot M.-T., Dorche C., Kisker C., Rajagopalan K.V.Hum. Mutat. 20:74-74(2002) A novel mutation in neonatal isolated sulphite oxidase deficiency.Lee H.F., Mak B.S., Chi C.S., Tsai C.R., Chen C.H., Shu S.G.Neuropediatrics 33:174-179(2002)

ncbi gi num :

74099694

ncbi acc num :

NP_000447.2

ncbi gb acc num :

NM_000456.2

uniprot acc num :

P51687

ncbi mol weight :

67.5kD

ncbi pathways :

Degradation Of Cysteine And Homocysteine Pathway (1270183); Metabolism Pathway (1269956); Metabolism Of Amino Acids And Derivatives Pathway (1270158); Sulfide Oxidation To Sulfate Pathway (1270184); Sulfur Amino Acid Metabolism Pathway (1270181); Sulfur Metabolism Pathway (83026); Sulfur Metabolism Pathway (417); Sulfite Oxidation Pathway (142428); Sulfite Oxidation IV Pathway (139568); Superpathway Of L-methionine Salvage And Degradation (139182)

ncbi summary :

Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008]

uniprot summary :

SUOX: Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD); also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age. Protein type: EC 1.8.3.1; Energy Metabolism - sulfur; Oxidoreductase; Mitochondrial. Chromosomal Location of Human Ortholog: 12q13.2. Cellular Component: cytosol; mitochondrial intermembrane space; mitochondrial matrix. Molecular Function: heme binding; molybdenum ion binding; molybdopterin cofactor binding; sulfite oxidase activity. Biological Process: nitrate assimilation; response to nutrient; sulfur amino acid catabolic process; sulfur amino acid metabolic process. Disease: Sulfocysteinuria

size1 :

0.05 mg (E-Coli)

price1 :

190 USD

size2 :

0.05 mg (Yeast)

price2 :

190

size3 :

0.2 mg (E-Coli)

price3 :

460

size4 :

0.2 mg (Yeast)

price4 :

460

size5 :

0.5 mg (E-Coli)

price5 :

750

more info or order :

MyBioSource product webpage