Recombinant Human HMGCL protein (His tag) (ab226881)
Key features and details
- Expression system: Baculovirus infected insect cells
- Purity: > 90% SDS-PAGE
- Endotoxin level: < 1.000 Eu/µg
- Tags: His tag C-Terminus
- Suitable for: SDS-PAGE
Description
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Product name
Recombinant Human HMGCL protein (His tag) -
Purity
> 90 % SDS-PAGE.
Affinity purified -
Endotoxin level
< 1.000 Eu/µg -
Expression system
Baculovirus infected insect cells -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MTLPKRVKIVEVGPRDGLQNEKNIVSTPVKIKLIDMLSEAGLSVIETTSF VSPKWVPQMGDHTEVLKGIQKFPGINYPVLTPNLKGFEAAVAAGAKEVVI FGAASELFTKKNINCSIEESFQRFDAILKAAQSANISVRGYVSCALGCPY EGKISPAKVAEVTKKFYSMGCYEISLGDTIGVGTPGIMKDMLSAVMQEVP LAALAVHCHDTYGQALANTLMALQMGVSVVDSSVAGLGGCPYAQGASGNL ATEDLVYMLEGLGIHTGVNLQKLLEAGNFICQALNRKTSSKVAQATCKLH HHHHH -
Predicted molecular weight
33 kDa including tags -
Amino acids
28 to 325 -
Tags
His tag C-Terminus -
Additional sequence information
This product is the mature full length protein from aa 28 to 325. The transit peptide is not included (NP_000182).
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab226881 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
SDS-PAGE
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Form
Liquid -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
pH: 7.40
Constituents: 0.02% DTT, 20% Glycerol (glycerin, glycerine), PBS
General Info
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Alternative names
- 3 hydroxy 3 methylglutaryl CoA lyase
- 3 hydroxy 3 methylglutaryl Coenzyme A lyase
- 3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase
see all -
Function
Involved in the catabolism of branched amino acids such as leucine. -
Tissue specificity
Fibroblasts, liver and lymphoblasts. -
Pathway
Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1. -
Involvement in disease
Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases. -
Sequence similarities
Belongs to the HMG-CoA lyase family. -
Cellular localization
Mitochondrion matrix. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human HMGCL protein (His tag) (ab226881)
15% SDS-PAGE analysis of 3 µg ab226881.
MW: 28-40 kDa (SDS-PAGE under reducing conditions).
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
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SDS download
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Datasheet download
References (0)
ab226881 has not yet been referenced specifically in any publications.