Metabolism Antibodies 1
Anti-ACADL Antibody (CAB1266)
- SKU:
- CAB1266
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
Antibody Name: | Anti-ACADL Antibody |
Antibody SKU: | CAB1266 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB IHC IF |
Reactivity: | Human, Mouse, Rat |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 31-210 of human ACADL (NP_001599.1). |
Application: | WB IHC IF |
Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
Reactivity: | Human, Mouse, Rat |
Positive Samples: | SH-SY5Y, Mouse liver, Mouse kidney, Mouse heart, Rat spinal cord |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 31-210 of human ACADL (NP_001599.1). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | GGEE RLET PSAK KLTD IGIR RIFS PEHD IFRK SVRK FFQE EVIP HHSE WEKA GEVS REVW EKAG KQGL LGVN IAEH LGGI GGDL YSAA IVWE EQAY SNCS GPGF SIHS GIVM SYIT NHGS EEQI KHFI PQMT AGKC IGAI AMTE PGAG SDLQ GIKT NAKK DGSD WILN GSKV FISN GSLS |
Gene ID: | 33 |
Uniprot: | P28330 |
Cellular Location: | Mitochondrion matrix |
Calculated MW: | 47kDa |
Observed MW: | 47kDa |
Synonyms: | ACADL, ACAD4, LCAD |
Background: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. |
UniProt Protein Function: | ACADL: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD). An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. |
UniProt Protein Details: | Protein type:Mitochondrial; Lipid Metabolism - fatty acid; EC 1.3.8.8; Oxidoreductase Chromosomal Location of Human Ortholog: 2q34 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; cytoplasm Molecular Function:palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding; long-chain-acyl-CoA dehydrogenase activity Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; cellular lipid catabolic process; carnitine catabolic process; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; long-chain fatty acid catabolic process; protein homotetramerization |
NCBI Summary: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008] |
UniProt Code: | P28330 |
NCBI GenInfo Identifier: | 223590148 |
NCBI Gene ID: | 33 |
NCBI Accession: | P28330.2 |
UniProt Related Accession: | P28330 |
Molecular Weight: | |
NCBI Full Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
NCBI Synonym Full Names: | acyl-CoA dehydrogenase long chain |
NCBI Official Symbol: | ACADL |
NCBI Official Synonym Symbols: | LCAD; ACAD4 |
NCBI Protein Information: | long-chain specific acyl-CoA dehydrogenase, mitochondrial |
UniProt Protein Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
Protein Family: | Long-chain specific acyl-CoA dehydrogenase |
UniProt Gene Name: | ACADL |
UniProt Entry Name: | ACADL_HUMAN |