ACADVL Antibody

Code CSB-PA001129LA01HU
Size US$166
Order now
Image
  • Western Blot
    Positive WB detected in: Hela whole cell lysate
    All lanes: ACADVL antibody at 2μg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 71, 69, 73 kDa
    Observed band size: 71 kDa

  • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA001129LA01HU at dilution of 1:100

  • Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA001129LA01HU at dilution of 1:100

  • Immunofluorescent analysis of A549 cells using CSB-PA001129LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

The Latest Promotion Free Antibody trial simple
Have Questions? Leave a Message or Start an on-line Chat

Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) ACADVL Polyclonal antibody
Uniprot No.
Target Names
ACADVL
Alternative Names
ACAD 6 antibody; ACAD6 antibody; ACADV_HUMAN antibody; Acadvl antibody; Acyl CoA dehydrogenase very long chain antibody; Acyl Coenzyme A dehydrogenase very long chain antibody; LCACD antibody; mitochondrial antibody; Very long chain specific acyl CoA dehydrogenase antibody; Very long chain specific acyl CoA dehydrogenase mitochondrial antibody; Very long-chain specific acyl-CoA dehydrogenase antibody; VLCAD antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Very long-chain specific acyl-CoA dehydrogenase, mitochondrial protein (403-497AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The ACADVL Antibody (Product code: CSB-PA001129LA01HU) is Non-conjugated. For ACADVL Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA001129LB01HU ACADVL Antibody, HRP conjugated ELISA
FITC CSB-PA001129LC01HU ACADVL Antibody, FITC conjugated
Biotin CSB-PA001129LD01HU ACADVL Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:20-1:200
IF 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

 Customer Reviews

There are currently no reviews for this product.

Submit a Review here

Target Background

Function
Very long-chain specific acyl-CoA dehydrogenase is one of the acyl-CoA dehydrogenases that catalyze the first step of mitochondrial fatty acid beta-oxidation, an aerobic process breaking down fatty acids into acetyl-CoA and allowing the production of energy from fats. The first step of fatty acid beta-oxidation consists in the removal of one hydrogen from C-2 and C-3 of the straight-chain fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, very long-chain specific acyl-CoA dehydrogenase acts specifically on acyl-CoAs with saturated 12 to 24 carbons long primary chains.
Gene References into Functions
  1. There are currently no biochemical markers for prediction of disease severity and for the need for treatment in VLCAD deficiency. Mutation analysis may offer predictive value but this may not be robust enough for a large proportion of those mutations that have not been previously reported in clinically affected patients. PMID: 27246109
  2. We retrospectively analyzed early outcomes for individuals who were diagnosed with VLCAD deficiency by NBS and describe initial presentations, diagnosis, clinical outcomes and treatment in a cohort of 52 individuals ages 1-18year. PMID: 27209629
  3. following variants should be considered likely pathogenic c.1273G > A (p.A425T), c.1001T > G (p.M334R), c.538G > A (p.A180T), c.640T > G (p.F214V), c.1076C > T (p.A359V), c.1019G > T (p.G340V), c.889_891delGAG (p.E297del), and c.1103A > C (p.Q368P); patients homozygous for the most common pathogenic variant, c.848T > C (p.V283A) can be expected to have a more benign clinical course PMID: 26385305
  4. 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. PMID: 24801231
  5. Case Report: missense mutation within the ACADVL gene responsible for very-long-chain acyl-CoA dehydrogenase deficiency and sudden infant death. PMID: 20107901
  6. These results emphasize the importance of functional investigation of abnormal NBS or clinical testing suggestive but not diagnostic of very-long-chain acyl-CoA dehydrogenase . PMID: 23480858
  7. 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 activity, but no mutations in the CPT2 gene can be identified. PMID: 23169530
  8. Identification of 2 VLCAD mutations leads to precautions in the management of the children with VLCAD deficiency. PMID: 21932095
  9. The expressions of LCHAD gene and protein are remarkably reduced in early onset severe preeclampsia and HELLP syndrome. PMID: 22093928
  10. Analyzed potential rhabdomyolysis-susceptibility genes (RYR 1, CPT II, VLCAD and CYP 2D6) from autopsy samples of methamphetamine abusers; no obvious relationship between the genetic mutations observed in this study and rhabdomyolysis was seen. PMID: 20952238
  11. Down regulation of ACADVL is associated with cervical squamous cell carcinoma. PMID: 20099975
  12. Missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase is associated with inborn errors of lipid metabolism. PMID: 20060901
  13. This study confirms that VLCAD deficiency, although being less frequent than CPT II deficiency, should be systematically considered in the differential diagnosis of exercise-induced rhabdomyolysis. PMID: 19327992
  14. Results suggest a novel regulatory mechanism for homeostatic VLCAD activity, whose dysregulation might be involved in the production of oxidative stress and in the pathogenesis of idiopathic pulmonary fibrosis. PMID: 19889959
  15. A new a unique mutation (IVS13+25G>A) is reported in a compound heterozygote carrying the 1748 C>T mutation in exon 18. PMID: 16464760
  16. the bacterial expression system developed here will significantly advance our understanding of both the clinical aspects of VLCAD deficiency and the basic biochemistry of the enzyme PMID: 17374501
  17. In asymptomatic mild VLCADdeficiency, a fat-reduced diet may not be necessary, whereas in later infancy and adolescence, strenuous physical exercise may require additional energy from medium-chain fat. PMID: 17457695
  18. Report the course of disease in a pair of monozygotic twin sisters. PMID: 17514507
  19. Bezafibrate, a widely prescribed hypolipidemic drug, cn be used for the correction of VLCAD deficiency and exemplifies the integration of molecular information in a therapeutic strategy PMID: 17999356
  20. Loss of heterozygosity on 17p13 and down-regulation of ACADVL can be used to discriminate adrenal cortex neoplasms from adrenocortical adenoma. PMID: 18156936

Show More

Hide All

Involvement in disease
Acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD)
Subcellular Location
Mitochondrion inner membrane; Peripheral membrane protein.; [Isoform 2]: Mitochondrion inner membrane; Peripheral membrane protein.
Protein Families
Acyl-CoA dehydrogenase family
Database Links

HGNC: 92

OMIM: 201475

KEGG: hsa:37

STRING: 9606.ENSP00000349297

UniGene: Hs.437178

icon of phone
Call us
301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)
icon of address
Address
7505 Fannin St., Ste 610, Room 322 (CUBIO Innovation Center), Houston, TX 77054, USA
icon of social media
Join us with

Subscribe newsletter

Leave a message

* To protect against spam, please pass the CAPTCHA test below.
CAPTCHA verification
© 2007-2024 CUSABIO TECHNOLOGY LLC All rights reserved. 鄂ICP备15011166号-1
Place an order now

I. Product details

*
*
*
*

II. Contact details

*
*

III. Ship To

*
*
*
*
*
*
*

IV. Bill To

*
*
*
*
*
*
*
*