TEL: +86 571 56623320 EMAIL: [email protected]
Product Name ACADL Chinese Name 酰基辅酶A脱氢酶长链抗体 Alias mitochondrial; ACAD4; ACADL; ACADL_HUMAN; Acyl Coenzyme A dehydrogenase long chain; FLJ94052; LCAD; Long chain acyl CoA dehydrogenase; Long-chain specific acyl-CoA dehydrogenase. literatures Specific References (1) | SL10150R has been referenced in 1 publications.[IF=4.213] Yang Jiao. et al. Lysine demethylation KDM5B downregulates SIRT3-mediated mitochondrial glucose and lipid metabolism in diabetic neuropathy. DIABETIC MED. 2022 Sep;:e14964 WB ; Mouse.Research Area Tumour Cell biology immunology transcriptional regulatory factor Mitochondrion Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 44kDa Cellular localization cytoplasmic Mitochondrion Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ACADL: 31-130/430 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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].
Subunit:
Homotetramer.
Subcellular Location:
Mitochondrion matrix.
DISEASE:
Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. 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.
Similarity:
Belongs to the acyl-CoA dehydrogenase family.
SWISS:
P28330
Gene ID:
33
Database links:
Entrez Gene: 11363 Mouse
Omim: 609576 Human
SwissProt: P28330 Human
SwissProt: P51174 Mouse
Unigene: 471277 Human
Unigene: 2445 Mouse
Unigene: 174 Rat
Scan Wechat Qrcode