Invitrogen

ACADVL Polyclonal Antibody

ACADVL Antibody in Immunocytochemistry (ICC/IF)

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ACADVL Antibody (PA5-29959) in ICC/IF

ACADVL Polyclonal Antibody detects ACADVL protein at mitochondria by immunofluorescent analysis. Sample: HeLa cells were fixed in ice-cold MeOH for 5 min. Green: ACADVL stained by ACADVL Polyclonal Antibody (Product # PA5-29959) diluted at 1:500. Blue: Fluoroshield with DAPI . Scale bar= 10 µm.

ACADVL Antibody in Immunohistochemistry (Paraffin) (IHC (P))

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产品信息

PA5-29959

应用

建议稀释比

已发表文章

免疫印迹 (WB)

1:500-1:3,000

查看4篇已发表文章 4篇已发表文章

免疫组化（石蜡） (IHC (P))

1:100-1:1,000

免疫细胞化学 (ICC/IF)

1:100-1:1,000

产品规格

种属反应

Human, Mouse

已发表种属

Human, Mouse

宿主/亚型

Rabbit / IgG

分类

Polyclonal

类型

Antibody

抗原

Recombinant fragment corresponding to a region within amino acids 18 and 341 of Human ACADVL

查看免疫原

偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

0.3 mg/mL

纯化类型

Antigen affinity chromatography

保存液

PBS, pH 7, with 20% glycerol, 1% BSA

内含物

0.025% ProClin 300

保存条件

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

运输条件

Wet ice

AB_2547433

产品详细信息

Recommended positive controls: H1299, HeLa, HepG2, mouse liver.

Predicted reactivity: Mouse (86%), Rat (88%), Zebrafish (81%), Bovine (92%).

Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

靶标信息

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.

仅用于科研。不用于诊断过程。未经明确授权不得转售。

Product References

A G1528C Hadha knock-in mouse model recapitulates aspects of human clinical phenotypes for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

Communications biology

Gaston G,Babcock S,Ryals R,Elizondo G,DeVine T,Wafai D,Packwood W,Holden S,Raber J,Lindner JR,Pennesi ME,Harding CO,Gillingham MB

PA5-29959 was used in Western Blot to report a CRISPR/Cas9 generated knock-in murine model of G1528C in Hadha that recapitulates aspects of the human LCHADD phenotype.

Tue Aug 29 00:00:00 UTC 2023

A G1528C Hadha knock-in mouse model recapitulates aspects of human clinical phenotypes for long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.

Communications biology

Gaston G,Babcock S,Ryals R,Elizondo G,DeVine T,Wafai D,Packwood W,Holden S,Raber J,Lindner JR,Pennesi ME,Harding CO,Gillingham MB

PA5-29959 was used in Western Blot to report a CRISPR/Cas9 generated knock-in murine model of G1528C in Hadha that recapitulates aspects of the human LCHADD phenotype.

Tue Aug 29 00:00:00 UTC 2023

Structural basis for defective membrane targeting of mutant enzyme in human VLCAD deficiency.

Nature communications

Prew MS,Camara CM,Botzanowski T,Moroco JA,Bloch NB,Levy HR,Seo HS,Dhe-Paganon S,Bird GH,Herce HD,Gygi MA,Escudero S,Wales TE,Engen JR,Walensky LD

PA5-29959 was used in Western Blotting to support a structural basis for VLCAD deficiency in patients with discrete mutations in an α-helical membrane-binding motif, resulting in pathologic enzyme mislocalization.

Mon Jun 27 00:00:00 UTC 2022

Dynamic Regulation of Long-Chain Fatty Acid Oxidation by a Noncanonical Interaction between the MCL-1 BH3 Helix and VLCAD.

Molecular cell

Escudero S,Zaganjor E,Lee S,Mill CP,Morgan AM,Crawford EB,Chen J,Wales TE,Mourtada R,Luccarelli J,Bird GH,Steidl U,Engen JR,Haigis MC,Opferman JT,Walensky LD