Rabbit Anti-ACOX1/FITC Conjugated antibody

Home > Product > Antibody > Rabbit Anti-ACOX1/FITC Conjugated antibody

ACOX1; ACOX1_HUMAN; AOX antibody Palmitoyl CoA oxidase; Palmitoyl-CoA oxidase; Peroxisomal acyl coenzyme A oxidase 1; Peroxisomal acyl-coenzyme A oxidase 1; SCOX; Straight chain acyl CoA oxidase; Straight-chain acyl-CoA oxidase.

Cat:

SL5021R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human ACOX1

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

74kDa

prev next

Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
Feedback Wall

background:
Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD); also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning.

Function:
Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.

Subcellular Location:
Peroxisome.

Tissue Specificity:
Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart.

DISEASE:
Defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal (Pseudo-NALD) [MIM:264470]; also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning.

Similarity:
Belongs to the acyl-CoA oxidase family.

Database links:

Entrez Gene: 51 Human

Entrez Gene: 11430 Mouse

Entrez Gene: 50681 Rat

Omim: 609751 Human

SwissProt: Q15067 Human

SwissProt: Q9R0H0 Mouse

SwissProt: P07872 Rat

Unigene: 464137 Human

Unigene: 356689 Mouse

Unigene: 31796 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

Product Feedback Wall