ACSF2 Antibody [F15E3]

Catalog No.: F4977

Application: Reactivity: Human

Lane 1: Jurkat

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代表番号: 045-509-1970|電子メール：sales@selleck.co.jp

使用情報

Dilution
WB1:2000-1:10000 FCM1:8000

Application
WB, FCM, ELISA

Source
Rabbit Monoclonal Antibody

Reactivity
Human

Storage Buffer
PBS, pH 7.2+50% Glycerol+0.05% BSA+0.01% NaN3

Storage (from the date of receipt)
-20°C (avoid freeze-thaw cycles), 2 years

Predicted MW Observed MW
68 kDa 51-71 kDa
^*なぜ予測分子量と実際の分子量が異なるのか？下記の原因により、実際の分子量が予測と異なる：タンパク質の翻訳後修飾（リン酸化/糖鎖付加），スプライシングバリアント，イソフォーム，相対的な電荷，ポリマー。

Datasheet & SDS

生物学的記述

Specificity
ACSF2 Antibody [F15E3] detects endogenous levels of total ACSF2 protein.

Clone
F15E3

Synonym(s)
ACSF2; Medium-chain acyl-CoA ligase ACSF2, mitochondrial; UNQ493/PRO1009

Background
ACSF2 (acyl-CoA synthetase family member 2) is a mitochondrial matrix enzyme that catalyzes the activation of medium-chain fatty acids into acyl-CoA thioesters, a crucial step for β-oxidation and maintaining lipid homeostasis. It possesses a canonical AMP-binding superfamily architecture, including an N-terminal mitochondrial targeting sequence that is cleaved upon import, an ATP-grasp domain responsible for Mg-ATP binding, an A10-like subdomain forming the substrate-binding cleft for fatty acid interaction, and a C-terminal dimerization domain that enables obligatory homodimerization. ACSF2 operates via a two-step ping-pong mechanism, where ATP thioesterification forms an acyl-AMP intermediate followed by nucleophilic displacement by the CoA thiol to generate acyl-CoA and AMP, with a marked specificity for medium-chain fatty acids. This enzyme supports mitochondrial β-oxidation by enabling CPT1-independent import of medium-chain fatty acids, bypassing carnitine shuttle limitations. Deficiency of ACSF2 leads to combined malonic and methylmalonic aciduria (CMAMMA), resulting in the accumulation of dicarboxylic acids due to impaired fatty acid catabolism and deficits in protein lipoylation, which are essential for mitochondrial enzyme complex stability. Somatic overexpression of ACSF2 is associated with non-alcoholic fatty liver disease and hepatocellular carcinoma through dysregulated hepatic VLDL secretion and increased lipid droplet accumulation.

Background

ACSF2 (acyl-CoA synthetase family member 2) is a mitochondrial matrix enzyme that catalyzes the activation of medium-chain fatty acids into acyl-CoA thioesters, a crucial step for β-oxidation and maintaining lipid homeostasis. It possesses a canonical AMP-binding superfamily architecture, including an N-terminal mitochondrial targeting sequence that is cleaved upon import, an ATP-grasp domain responsible for Mg-ATP binding, an A10-like subdomain forming the substrate-binding cleft for fatty acid interaction, and a C-terminal dimerization domain that enables obligatory homodimerization. ACSF2 operates via a two-step ping-pong mechanism, where ATP thioesterification forms an acyl-AMP intermediate followed by nucleophilic displacement by the CoA thiol to generate acyl-CoA and AMP, with a marked specificity for medium-chain fatty acids. This enzyme supports mitochondrial β-oxidation by enabling CPT1-independent import of medium-chain fatty acids, bypassing carnitine shuttle limitations. Deficiency of ACSF2 leads to combined malonic and methylmalonic aciduria (CMAMMA), resulting in the accumulation of dicarboxylic acids due to impaired fatty acid catabolism and deficits in protein lipoylation, which are essential for mitochondrial enzyme complex stability. Somatic overexpression of ACSF2 is associated with non-alcoholic fatty liver disease and hepatocellular carcinoma through dysregulated hepatic VLDL secretion and increased lipid droplet accumulation.

References
[1] Zhang F, et al. Front Genet. 2022 Sep 7;13:980463. [2] Maiguel D, et al. bioRxiv (2022): 2022-03.

PVDFメンブレン孔径参考表

Protein Size (kDa)	PVDF Transfer Membrane Pore Size (μm)
< 10	0.22
10-20	0.22
20-30	0.45
30-45	0.45
45-70	0.45
80-100	0.45
100-140	0.45
> 140	0.45

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Protein Size (kDa)	Separating Gel Percentage
4-40	20%
12-45	15%
10-70	12.5%
15-100	10%
25-100	8%
60-210	5%