Recombinant Human 3-Hydroxyisobutyryl-CoA Hydrolase | MyBioSource MBS144549 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Recombinant Human 3-Hydroxyisobutyryl-CoA Hydrolase

catalog :

MBS144549

quantity :

0.002 mg

price :

140 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS144549

products type :

Recombinant Protein

products full name :

Recombinant Human 3-Hydroxyisobutyryl-CoA Hydrolase

products short name :

[3-Hydroxyisobutyryl-CoA Hydrolase]

products name syn :

[HIBCH Human; 3-Hydroxyisobutyryl-CoA Hydrolase Human Recombinant; 3-hydroxyisobutyryl-coenzyme A hydrolase mitochondrial; HIBYL-CoA-H; HIB-CoA hydrolase; EC 3.1.2.4]

other names :

[3-hydroxyisobutyryl-CoA hydrolase, mitochondrial isoform 1; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial; 3-hydroxyisobutyryl-Coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; HIB-CoA hydrolase; HIBYL-CoA-H]

products gene name :

[HIBCH]

other gene names :

[HIBCH; HIBCH; HIBYLCOAH; HIB-CoA hydrolase; HIBYL-CoA-H]

uniprot entry name :

HIBCH_HUMAN

sequence length :

386

sequence :

MGSSHHHHHH SSGLVPRGSH MGSHM DAAEE VLLEKKGCTG VITLNRPKFL NALTLNMIRQ IYPQLKKWEQ DPETFLIIIK GAGGKAFCAG GDIRVISEAE KAKQKIAPVF FREEYMLNNA VGSCQKPYVA LIHGITMGGG VGLSVHGQFR VATEKCLFAM PETAIGLFPD VGGGYFLPRL QGKLGYFLAL TGFRLKGRDV YRAGIATHFV DSEKLAMLEE DLLALKSPSK ENIASVLENY HTESKIDRDK SFILEEHMDK INSCFSANTV EEIIENLQQD GSSFALEQLK VINKMSPTSL KITLRQLMEG SSKTLQEVLT MEYRLSQACM RGHDFHEGVR AVLIDKDQSP KWKPADLKEV TEEDLNNHFK SLGSSDLKF.

purity :

Greater than 90% as determined by SDS-PAGE.

form :

The HIBCH solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 200mM Nacl, 1mM DTT and 10% glycerol.

storage stability :

Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.

other info1 :

Source: E.coli

other info2 :

Physical Appearance: Sterile filtered colorless solution

products categories :

ENZYMES; Enzymes; Hydrolase

products description :

HIBCH Recombinant produced in E. coli is a single polypeptide chain containing 379 amino acids (33-386) and having a molecular mass of 42.1kDa. HIBCH is fused to a 25 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

ncbi gi num :

37594471

ncbi acc num :

NP_055177.2

ncbi gb acc num :

NM_014362.3

uniprot acc num :

Q6NVY1

ncbi pathways :

Branched-chain Amino Acid Catabolism Pathway (106179); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (413478); Malonate Semialdehyde Pathway, Propanoyl-CoA = Acetyl-CoA (468206); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Propanoate Metabolism Pathway (83004); Propanoate Metabolism Pathway (387)

ncbi summary :

This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]

uniprot summary :

HIBCH: Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD); also known as deficiency of beta- hydroxyisobutyryl CoA deacylase or methacrylic aciduria. The enzyme defect results in accumulation of methacrylyl-CoA, a highly reactive compound, which readily undergoes addition reactions with free sulfhydryl groups. Affected individuals showed delayed development of motor skills, hypotonia, initial poor feeding, and a deterioration in neurological function during first stages of life. Belongs to the enoyl-CoA hydratase/isomerase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Mitochondrial; Other Amino Acids Metabolism - beta-alanine; EC 3.1.2.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; Hydrolase. Chromosomal Location of Human Ortholog: 2q32.2. Cellular Component: mitochondrial matrix. Molecular Function: 3-hydroxyisobutyryl-CoA hydrolase activity. Biological Process: valine catabolic process; branched chain family amino acid catabolic process. Disease: Beta-hydroxyisobutyryl Coa Deacylase Deficiency

size1 :

0.002 mg

price1 :

140 USD

size2 :

0.01 mg

price2 :

205

size3 :

1 mg

price3 :

5015

more info or order :

MyBioSource product webpage