Anti-HMGCL Antibody

$99.00$319.00

Reactivity: Human,Rat
Applications: ELISA,WB,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-HMGCL Antibody (CSB-PA010563ESR2HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Hydroxymethylglutaryl-CoA lyase Antibody,mitochondrial Antibody,HL Antibody,HMG-CoA lyase Antibody,3-hydroxy-3-methylglutarate-CoA lyase Antibody,HMGCL Antibody

More alternative names for the antibody
3 hydroxy 3 methylglutaryl CoA lyase antibody|3 hydroxy 3 methylglutaryl Coenzyme A lyase antibody|3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase (hydroxymethylglutaricaciduria) antibody|3 hydroxymethyl 3 methylglutaryl Coenzyme A lyase antibody|3-hydroxy-3-methylglutarate-CoA lyase antibody|HL antibody|HMG CoA lyase antibody|HMG CoA Lyase Deficiency antibody|HMG-CoA lyase antibody|HMGCL antibody|HMGCL_HUMAN antibody|Hydroxymethylglutaricaciduria antibody|Hydroxymethylglutaryl CoA lyase antibody|Hydroxymethylglutaryl CoA lyase mitochondrial antibody|Hydroxymethylglutaryl-CoA lyase antibody|Mitochondrial 3 hydroxy 3 methylglutaryl CoA lyase antibody|mitochondrial antibody|MS725 antibody|OTTHUMP00000044830 antibody
Anti-HMGCL antibody [EPR15550] – N-terminal (ab187648)
Close sc-100548|sc-101973|sc-133661|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-HMGCL Antibody

Catalogue No.

CSB-PA010563ESR2HU

Reactivity

Human, Rat

Immunogen

Recombinant human Hydroxymethylglutaryl-CoA lyase, mitochondrial protein (1-325AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

34 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:3155( Human), Omim:246450( Human), Omim:613898( Human), SwissProt:P35914( Human), Unigene:533444( Human)

Protein function

Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism. .

Protein tissue specificity

Highest expression in liver. Expressed in pancreas, kidney, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%). .

Involvement in disease

3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD) [MIM:246450]: 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Protein sequence and domain

Belongs to the HMG-CoA lyase family.

Protein cellular localization

Mitochondrion matrix. Peroxisome. Note=Unprocessed form is peroxisomal.

Research area

All research areas>Synthesis and Degradation>HMGCL
(View all antibody categories related to Synthesis and Degradation)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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