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Acyl-CoA Dehydrogenase, Very Long Chain Proteins (ACADVL)

The protein encoded by ACADVL is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. Additionally we are shipping ACADVL Antibodies (71) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
ACADVL 37 P49748
ACADVL 25363 P45953
ACADVL 11370 P50544
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Top ACADVL Proteins at antibodies-online.com

Showing 9 out of 13 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Wheat germ Human GST tag 2 μg Log in to see 9 Days
$333.33
Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Escherichia coli (E. coli) Human His tag 50 μg Log in to see 7 to 8 Days
$407.00
Details
Yeast Rat His tag   1 mg Log in to see 56 to 66 Days
$3,774.83
Details
HOST_Escherichia coli (E. coli) Human His tag   1 mg Log in to see 3 to 4 Days
$5,769.50
Details
HOST_Escherichia coli (E. coli) Human Un-conjugated   50 μg Log in to see 3 to 4 Days
$467.86
Details
HOST_Human Human Un-conjugated   20 μg Log in to see 9 to 11 Days
$785.40
Details
Human Un-conjugated   2 μg Log in to see 6 Days
$168.54
Details

ACADVL Proteins by Origin and Source

Origin Expressed in Conjugate
Human , ,
,
Rat (Rattus)

Mouse (Murine)

Top referenced ACADVL Proteins

  1. Human ACADVL Protein expressed in Escherichia coli (E. coli) - ABIN667811 : Smelt, Poorthuis, Onkenhout, Scholte, Andresen, van Duinen, Gregersen, Wintzen: Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset. in Annals of neurology 1998 (PubMed)
    Show all 2 references for ABIN667811

  2. Human ACADVL Protein expressed in Wheat germ - ABIN1305595 : Muroya, Ito, Rong, Takashima, Ito, Cao, Nakamura, Joh, Kohzuki: Disorder of fatty acid metabolism in the kidney of PAN-induced nephrotic rats. in American journal of physiology. Renal physiology 2012 (PubMed)

More Proteins for Acyl-CoA Dehydrogenase, Very Long Chain (ACADVL) Interaction Partners

Human Acyl-CoA Dehydrogenase, Very Long Chain (ACADVL) interaction partners

  1. 11 mutations in ACADVL gene in 7 patients, 7 reported (p.S22X, p.W427X, p.A213T, p.G222R, p.R450H, c.296-297delCA, c.1605+1G>T), 4 novel (p.S72F, p.Q100X, p.M437T, p.D466Y). p.R450H and p.D466Y (14.28%, 2/14 alleles) mutations identified in 2 alleles.

  2. Case Report: missense mutation within the ACADVL gene responsible for very-long-chain acyl-CoA dehydrogenase deficiency and sudden infant death.

  3. These results emphasize the importance of functional investigation of abnormal NBS (show NBN Proteins) or clinical testing suggestive but not diagnostic of very-long-chain acyl-CoA dehydrogenase .

  4. These findings support the importance of considering that mutations may be present in the ACADVL gene when a significant partial deficiency is found in CPTII (show CPT2 Proteins) activity, but no mutations in the CPT2 (show CPT2 Proteins) gene can be identified.

  5. Identification of 2 VLCAD mutations leads to precautions in the management of the children with VLCAD deficiency.

  6. The expressions of LCHAD (show HADHA Proteins) gene and protein are remarkably reduced in early onset severe preeclampsia and HELLP syndrome.

  7. Analyzed potential rhabdomyolysis-susceptibility genes (RYR 1, CPT II (show CPT2 Proteins), VLCAD and CYP (show PPIG Proteins) 2D6) from autopsy samples of methamphetamine abusers; no obvious relationship between the genetic mutations observed in this study and rhabdomyolysis was seen.

  8. Down regulation of ACADVL is associated with cervical squamous cell carcinoma.

  9. Missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase is associated with inborn errors of lipid metabolism.

  10. This study confirms that VLCAD deficiency, although being less frequent than CPT II (show CPT2 Proteins) deficiency, should be systematically considered in the differential diagnosis of exercise-induced rhabdomyolysis.

Cow (Bovine) Acyl-CoA Dehydrogenase, Very Long Chain (ACADVL) interaction partners

  1. Bovine ACADVL gene had a significant effect on chest width (P<0.05), chest depth (P<0.05), and hip width (P<0.05) in the Qinchuan breed.

Mouse (Murine) Acyl-CoA Dehydrogenase, Very Long Chain (ACADVL) interaction partners

  1. observed strong upregulation of peroxisomal beta-oxidation in VLCAD(-/-) mice

  2. SIRT3 (show SIRT3 Proteins) and SIRT5 (show SIRT5 Proteins) regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase

  3. Studies conducted with permeabilized mitochondria and different chain length acyl-CoA (show GNPAT Proteins) derivatives suggest that VLCAD is also a source of reactive oxygen species production in mitochondria of high fat diet animals.

  4. We demonstrate here that both dietary interventions with respect to the fat content of the diet reverse endogenous compensatory mechanisms in muscle that have evolved in VLCAD(-/-) mice resulting in pronounced energy deficiency

  5. VLCAD(-/-) mice develop tissue-specific strategies to compensate deficiency of VLCAD either by induction of other mitochondrial acyl-CoA (show GNPAT Proteins) dehydrogenases or by enhancement of glucose oxidation.

  6. Report a longer QTc interval and lipid alterations in VLCAD null mice.

  7. Four VLCAD-/- deficient mice died unexpectedly on the treadmill during the early stages of training. The VLCAD-/- deficient mice that survived adapted to the aerobic interval training similarly to the non-deficient mice.

  8. Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice

  9. Data show that in VLCAD knockout mice fed a long-chain triglyceride diet, fasting results in accumulation of liver lipids, hepatopathy and upregulation of peroxisomal and microsomal oxidation pathways as well as antioxidant enzyme activities and TBARS.

  10. medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice

ACADVL Protein Profile

Protein Summary

The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms.

Gene names and symbols associated with ACADVL

  • acyl-CoA dehydrogenase, very long chain (ACADVL)
  • acyl-CoA dehydrogenase, very long chain (Acadvl)
  • acyl-Coenzyme A dehydrogenase, very long chain (acadvl)
  • acyl-Coenzyme A dehydrogenase, very long chain (Acadvl)
  • ACAD6 protein
  • fb52d04 protein
  • LCACD protein
  • vlcad protein
  • wu:fb52d04 protein
  • wu:fc75e01 protein
  • zgc:64067 protein

Protein level used designations for ACADVL

acyl-Coenzyme A dehydrogenase, very long chain , very long-chain specific acyl-CoA dehydrogenase, mitochondrial , VLCAD , acyl-coenzyme A dehydrogenase, very long chain , VLCAD very-long-chain acyl-CoA dehydrogenase , Very long chain Acyl-Coa dehydrogenase , vlcad , MVLCAD

GENE ID SPECIES
100061583 Equus caballus
37 Homo sapiens
489463 Canis lupus familiaris
282130 Bos taurus
25363 Rattus norvegicus
573723 Danio rerio
11370 Mus musculus
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