Human arylsulfatase A (ASA) ELISA Kit | MyBioSource MBS265376 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human arylsulfatase A (ASA) ELISA Kit

catalog :

MBS265376

quantity :

48-Strip-Wells

price :

275 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS265376

products type :

ELISA Kit

products full name :

Human arylsulfatase A (ASA) ELISA Kit

products short name :

arylsulfatase A (ASA)

other names :

arylsulfatase A isoform a; Arylsulfatase A; arylsulfatase A; ASA; cerebroside-sulfatase; arylsulfatase A; Cerebroside-sulfataseCleaved into the following 2 chains:Arylsulfatase A component B; Arylsulfatase A component C

products gene name :

ASA

other gene names :

ARSA; ARSA; MLD; ASA

uniprot entry name :

ARSA_HUMAN

reactivity :

Human

sequence length :

509

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Human serum, plasma or Cell Culture Supernatant and organizations in the natural and recombinant ASA concentration. Assay Type: Sandwich. Detection Range: 20 ng/ml-0.312 ng/ml

products description :

Principle of the Assay: This experiment use double-sandwich elisa technique and the ELISA Kit provided is typical. The pre-coated antibody is human ASA monoclonal antibody and the detecting antibody is polyclonal antibody with biotin labeled. Samples and biotin labeling antibody are added into ELISA plate wells and washed out with PBS or TBS. Then Avidin-peroxidase conjugates are added to ELISA wells in order; Use TMB substrate for coloring after reactant thoroughly washed out by PBS or TBS. TMB turns into blue in peroxidase catalytic and finally turns into yellow under the action of acid. The color depth and the testing factors in samples are positively correlated.

ncbi gi num :

313569797

ncbi acc num :

NP_001078896.2

ncbi gb acc num :

NM_001085427.2

ncbi mol weight :

44,881 Da

ncbi pathways :

Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Metabolism Of Proteins Pathway (106230); PTM: Gamma Carboxylation, Hypusine Formation And Arylsulfatase Activation Pathway (106231); Post-translational Protein Modification Pathway (161012); Sphingolipid Metabolism Pathway (82994); Sphingolipid Metabolism Pathway (119543)

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; Lipid Metabolism - sphingolipid; EC 3.1.6.8. Chromosomal Location of Human Ortholog: 22q13.33. Cellular Component: extracellular space; lysosomal lumen; extrinsic to external side of plasma membrane; lysosome; endoplasmic reticulum lumen; acrosome; integral to membrane; endosome. Molecular Function: arylsulfatase activity; sulfuric ester hydrolase activity; calcium ion binding; cerebroside-sulfatase activity. Biological Process: cellular protein metabolic process; central nervous system development; sphingolipid metabolic process; response to ethanol; binding of sperm to zona pellucida; response to estrogen stimulus; response to methylmercury; autophagy; glycosphingolipid metabolic process; post-translational protein modification; response to nutrient; response to pH. Disease: Metachromatic Leukodystrophy

size1 :

48-Strip-Wells

price1 :

275 USD

size2 :

96-Strip-Wells

price2 :

420

more info or order :

MyBioSource product webpage