Anti-ARSA antibody (AA24929)

$187.00


Alternative names Arylsulfatase A, ASA, EC 3.1.6.8, Cerebroside-sulfatase, ARSA, ARSA_HUMAN, P15289, 410
Source 507?509?
Species Reactivity Human, Mouse, Rat
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ARSA antibody (AA24929)
Catalog number AA24929
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Arylsulfatase A antibody, ASA antibody, EC 3.1.6.8 antibody, Cerebroside-sulfatase antibody, ARSA antibody, ARSA_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ARSA
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name ARSA
SwissProt ID Link P15289
Function Hydrolyzes cerebroside sulfate.
Subcellular Location Lysosome.
Involvement in Disease Leukodystrophy metachromatic: A leukodystrophy due to a lysosomal storage defect. 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. The disease is caused by mutations affecting the gene represented in this entry. Multiple sulfatase deficiency: 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. The protein represented in this entry is involved in disease pathogenesis. 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.
Post-Translational Modification The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. This post-translational modification is severely defective in multiple sulfatase deficiency (MSD).
Subunit Structure Homodimer at neutral pH and homooctamer at acidic pH. Exists both as a single chain of 58 kDa (component A) or as a chain of 50 kDa (component B) linked by disulfide bond(s) to a 7 kDa chain (component C). Interacts with SUMF1.
Sequence Similarities Belongs to the sulfatase family.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.