ARSA Rabbit Polyclonal | MyBioSource MBS766842 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

ARSA Rabbit Polyclonal

catalog :

MBS766842

quantity :

0.1 mg

price :

265 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, enzyme immunoassay

more info or order :

MyBioSource product webpage

image

image 1 :

Immunohistochemistry of paraffin-embedded human cervical cancer using MBS766842 (ARSA antibody) at dilution of 1:50

image 2 :

mouse testis tissue were subjected to SDS PAGE followed by western blot with MBS766842 (ARSA antibody) at dilution of 1:1000

product information

catalog number :

MBS766842

products type :

Antibody

products full name :

ARSA Rabbit Polyclonal

products short name :

[ARSA]

products name syn :

[ARSA, arylsulfatase A, ASA, Cerebroside sulfatase, MLD]

other names :

[ARSA; Arylsulfatase A; arylsulfatase A; arylsulfatase A; Cerebroside-sulfatase]

products gene name :

[ARSA]

other gene names :

[ARSA; ARSA; MLD; ASA]

uniprot entry name :

ARSA_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse, Rat. Other species are not tested.

sequence length :

509

specificity :

Please decide specifity by homology.

purity :

>=95% as determined by SDS-PAGE. Immunogen Affinity Purified

form :

Liquid

storage stability :

-20°C for 24 months (Avoid repeated freeze / thaw cycles.)

tested application :

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

app notes :

WB: 1:500-1:2000. IHC: 1:20-1:200

image1 heading :

Immunohistochemistry (IHC)

image2 heading :

SDS-PAGE

other info1 :

Immunogen: Arylsulfatase A

other info2 :

Calculated MW: 56kd. Buffer: PBS with 0.02% sodium azide and 50% glycerol pH 7.3

products description :

Hydrolyzes cerebroside sulfate

ncbi gi num :

47678297

ncbi acc num :

CAG30269.1

uniprot acc num :

P15289

ncbi pathways :

Gamma Carboxylation, Hypusine Formation And Arylsulfatase Activation Pathway (1268702); Glycosphingolipid Metabolism Pathway (1270099); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Metabolism Of Proteins Pathway (1268677); Post-translational Protein Modification Pathway (1268701); Sphingolipid Metabolism Pathway (82994); Sphingolipid Metabolism Pathway (1270097)

ncbi summary :

The protein encoded by this gene hydrolyzes cerebroside sulfate to cerebroside and sulfate. Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimately death. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Dec 2010]

uniprot summary :

ARSA: Hydrolyzes cerebroside sulfate. Defects in ARSA are a cause of leukodystrophy metachromatic (MLD). MLD is a disease due to a lysosomal storage defect. It is characterized by intralysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues, with a diffuse loss of myelin in the central nervous system. Progressive demyelination causes a variety of neurological symptoms, including gait disturbances, ataxias, optical atrophy, dementia, seizures, and spastic tetraparesis. Three forms of the disease can be distinguished according to the age at onset: late- infantile, juvenile and adult. Arylsulfatase A activity is defective in multiple sulfatase deficiency (MSD). A clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Arylsulfatase A activity is impaired in multiple sulfatase deficiency due to mutations in SUMF1. SUMF1 mutations result in defective post-translational modification of ARSA at residue Cys- 69 that is not converted to 3-oxoalanine. Belongs to the sulfatase family. Protein type: Lipid Metabolism - sphingolipid; EC 3.1.6.8; Hydrolase. Chromosomal Location of Human Ortholog: 22q13.33. Cellular Component: endoplasmic reticulum lumen; lysosomal lumen; lysosome. Molecular Function: arylsulfatase activity; calcium ion binding; cerebroside-sulfatase activity; protein binding; sulfuric ester hydrolase activity. Biological Process: glycosphingolipid metabolic process; post-translational protein modification. Disease: Metachromatic Leukodystrophy

size1 :

0.1 mg

price1 :

265 USD

more info or order :

MyBioSource product webpage