Recombinant Human HIBCH protein (ab124585)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE, MS
Description
-
Product name
Recombinant Human HIBCH protein -
Purity
> 90 % SDS-PAGE.
ab124585 is purified using conventional chromatography techniques. -
Expression system
Escherichia coli -
Accession
-
Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
-
Species
Human -
Sequence
MGSSHHHHHH SSGLVPRGSH MGSHMDAAEE 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 -
Predicted molecular weight
42 kDa including tags -
Amino acids
33 to 386 -
Tags
His tag N-Terminus
-
Associated products
-
Related Products
Specifications
Our Abpromise guarantee covers the use of ab124585 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
-
Applications
SDS-PAGE
Mass Spectrometry
-
Mass spectrometry
MALDI-TOF -
Form
Liquid -
Concentration information loading...
Preparation and Storage
-
Stability and Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
pH: 8.00
Constituents: 0.02% DTT, 0.32% Tris HCl, 10% Glycerol (glycerin, glycerine), 1.17% Sodium chloride
General Info
-
Alternative names
- 3 hydroxyisobutyryl Coenzyme A hydrolase
- 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial
- 3-hydroxyisobutyryl-CoA hydrolase
see all -
Function
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. -
Tissue specificity
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung. -
Pathway
Amino-acid degradation; L-valine degradation. -
Involvement in disease
Defects in HIBCH are the cause of HIBCH deficiency (HIBCHD) [MIM:250620]; 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. -
Sequence similarities
Belongs to the enoyl-CoA hydratase/isomerase family. -
Cellular localization
Mitochondrion. - Information by UniProt
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
-
SDS download
-
Datasheet download
References (0)
ab124585 has not yet been referenced specifically in any publications.