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HMGCL (3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria))

HMGCL (3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (hydroxymethylglutaricaciduria))

Item no.	18-003-44494
Manufacturer	GENWAY
Amount	0.05 mg
Category	Antibodies Western Blot Primary Antibodies
Type	Antibody
Applications	WB
Specific against	other
ECLASS 10.1	32160702
ECLASS 11.0	32160702
UNSPSC	12352203
Alias	GWB-645BA7
Similar products	18-003-44494
Available
Genway ID:	GWB-645BA7
Antigen Specificity:	Polyclonal antibody produced in rabbits immunized with a synthetic peptide corresponding to a region of Human HMGCL.
ELISA Titre:	1:312500
Category:	Signal Protein
Note:	Suggested starting concentrations are provided. Optimal dilutions should be determined by end-user. Differences in calculated versus apparent molecular weight may be due to post-translational modifications or protein hydrophobicity. The function remains unknown.
Catalytic Activity:	(S)-3-hydroxy-3-methylglutaryl-CoA = acetyl-CoA + acetoacetate.
Pathway:	Ketogenesis and leucine catabolism; final step.
Subunit:	Homodimer.
Subcellular Location:	Mitochondrion matrix.
Tissue Specificity:	Fibroblasts liver and lymphoblasts.
Disease:	Defects in HMGCL are the cause of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMG-CoA lyase deficiency) [MIM:246450]; also known as hydroxymethylglutaricaciduria or HL deficiency. It is an autosomal recessive disease which can lead to hypoglycemia and coma.
Similarity:	Belongs to the HMG-CoA lyase family. Fu. Z. . (2006) J. Biol. Chem. 281 (11). 7526-7532.

Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.

All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.

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Amount: 0.05 mg

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Delivery expected until 8/28/2025

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