Goat anti-Arylsulfatase A Antibody | MyBioSource MBS421522 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-Arylsulfatase A Antibody

catalog :

MBS421522

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS421522

products type :

Antibody

products full name :

Goat anti-Arylsulfatase A Antibody

products short name :

Arylsulfatase A

products name syn :

ARSA; arylsulfatase A; MLD; cerebroside-sulfatase; cerebroside 3-sulfatase; ARSA antibody; arylsulfatase A antibody; MLD antibody; cerebroside-sulfatase antibody; cerebroside 3-sulfatase antibody; Arylsulfatase A; Arylsulfatase A

other names :

arylsulfatase A isoform a; 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

host :

Goat

reactivity :

Tested: Mouse; Expected from sequence similarity: Human, Mouse, Rat

sequence length :

507

sequence :

YDLSKDPGENYN

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 32000. Western Blot: Approx 55kDa band observed in Mouse Testis lysates (calculated MW of 53.6kDa according to human NP_000478.2 and 53.7kDa according to Mouse NP_033843.2). Recommended concentration: 0.5-1.5ug/ml.

other info1 :

Immunogen: Peptide with sequence C-YDLSKDPGENYN, from the internal region of the protein sequence according to NP_000478.2. Epitope: Internal region

ncbi gi num :

6005990

ncbi acc num :

NP_000478.2

ncbi gb acc num :

NM_000487.4

ncbi mol weight :

44,881 Da

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: Hydrolase; EC 3.1.6.8; Lipid Metabolism - sphingolipid. 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 :

300 USD

more info or order :

MyBioSource product webpage