HMGCL Antibody (PACO44348)
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주문- SKU:
- PACO44348
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | HMGCL Antibody (PACO44348) |
Antibody SKU: | PACO44348 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200 |
Species Reactivity: | Human, Rat |
Immunogen: | Recombinant Human Hydroxymethylglutaryl-CoA lyase, mitochondrial protein (1-325AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot. All lanes: HMGCL antibody at 0.8µg/ml + Rat liver tissue. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 35, 27, 21 kDa. Observed band size: 35 kDa.. |
![]() | Immunohistochemistry of paraffin-embedded human liver tissue using PACO44348 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human testis tissue using PACO44348 at dilution of 1:100. |
Background: | 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. |
Synonyms: | Hydroxymethylglutaryl-CoA lyase, mitochondrial (HL) (HMG-CoA lyase) (EC 4.1.3.4) (3-hydroxy-3-methylglutarate-CoA lyase), HMGCL |
UniProt Protein Function: | HMGCL: Key enzyme in ketogenesis (ketone body formation). Terminal step in leucine catabolism. Defects in HMGCL are the cause of 3-hydroxy-3- methylglutaryl-CoA lyase deficiency (HMGCLD); 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. Belongs to the HMG-CoA lyase family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Carbohydrate Metabolism - butanoate; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 4.1.3.4; Lyase; Lipid Metabolism - synthesis and degradation of ketone bodies Chromosomal Location of Human Ortholog: 1p36.1-p35 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; peroxisome Molecular Function:hydroxymethylglutaryl-CoA lyase activity; protein homodimerization activity; acyl-CoA binding; carboxylic acid binding; manganese ion binding; metal ion binding; magnesium ion binding; receptor binding Biological Process: response to starvation; mitochondrion organization and biogenesis; leucine catabolic process; acyl-CoA metabolic process; ketone body biosynthetic process; ketone body metabolic process; cellular lipid metabolic process; liver development; protein tetramerization; response to nutrient Disease: 3-hydroxy-3-methylglutaryl-coa Lyase Deficiency |
NCBI Summary: | The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009] |
UniProt Code: | P35914 |
NCBI GenInfo Identifier: | 24418852 |
NCBI Gene ID: | 3155 |
NCBI Accession: | P35914.2 |
UniProt Related Accession: | P35914 |
Molecular Weight: | Observed: 34 kDaPredicted: 35 kDa |
NCBI Full Name: | Hydroxymethylglutaryl-CoA lyase, mitochondrial |
NCBI Synonym Full Names: | 3-hydroxy-3-methylglutaryl-CoA lyase |
NCBI Official Symbol: | HMGCL |
NCBI Official Synonym Symbols: | HL |
NCBI Protein Information: | hydroxymethylglutaryl-CoA lyase, mitochondrial |
UniProt Protein Name: | Hydroxymethylglutaryl-CoA lyase, mitochondrial |
UniProt Synonym Protein Names: | 3-hydroxy-3-methylglutarate-CoA lyase |
UniProt Gene Name: | HMGCL |
UniProt Entry Name: | HMGCL_HUMAN |