Arylsulfatase A; arsA; ARSA_HUMAN; arylsulfatase A; Arylsulfatase A component C; As 2; AS A; As2; ASA; AW212749; C230037L18Rik; Cerebroside-sulfatase; metachromatic leucodystrophy; MGC125207; MLD; OTTHUMP00000196546; OTTHUMP00000196548; TISP73; arylsulfatase A

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Immunogen Affinity Purified

Lyophilized. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquoted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-ARSA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: Rabbit IgG polyclonal antibody for Arylsulfatase A(ARSA) detection. Tested with WB, IHC-P in Human;Mouse;Rat.

Background: Arylsulfatase A (ARSA) is an enzyme that breaks down sulfatides, namely cerebroside 3-sulfate intocerebroside and sulfate. In humans, arylsulfatase A is encoded by the ARSA gene. ARSA is mapped to 22q13.33. 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.

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

Western Blot Concentration: 0.1-0.5ug/ml
Immunohistochemistry (IHC) Paraffin Concentration: 0.5-1ug/ml

Figure 1. Western blot analysis of ARSA using anti-ARSA antibody (MBS177695).
Electrophoresis was performed on a 5-20% SDS-PAGE gel at 70V (Stacking gel) / 90V (Resolving gel) for 2-3 hours. The sample well of each lane was loaded with 50ug of sample under reducing conditions.
Lane 1: Rat Testis Tissue Lysate,
Lane 2: Rat Pancreas Tissue Lysate,
Lane 3: Rat Skeletal Muscle Tissue Lysate,
Lane 4: Mouse Kidney Tissue Lysate,
Lane 5: MCF-7 Whole Cell Lysate.
After Electrophoresis, proteins were transferred to a Nitrocellulose membrane at 150mA for 50-90 minutes. Blocked the membrane with 5% Non-fat Milk/ TBS for 1.5 hour at RT. The membrane was incubated with rabbit anti-ARSA antigen affinity purified polyclonal antibody at 0.5ug/mL overnight at 4 degree C, then washed with TBS-0.1%Tween 3 times with 5 minutes each and probed with a goat anti-rabbit IgG-HRP secondary antibody at a dilution of 1:10000 for 1.5 hour at RT. The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit with Tanon 5200 system. A specific band was detected for ARSA at approximately 54KD. The expected band size for ARSA is at 54KD.

Figure 2. IHC analysis of ARSA using anti-ARSA antibody (MBS177695).
ARSA was detected in paraffin-embedded section of Rat Lung Tissue. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1ug/ml rabbit anti-ARSA Antibody (MBS177695) overnight at 4 degree C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37 degree C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC) with DAB as the chromogen.

Figure 3. IHC analysis of ARSA using anti-ARSA antibody (MBS177695).
ARSA was detected in paraffin-embedded section of Human Mammary Cancer Tissue. Heat mediated antigen retrieval was performed in citrate buffer (pH6, epitope retrieval solution) for 20 mins. The tissue section was blocked with 10% goat serum. The tissue section was then incubated with 1ug/ml rabbit anti-ARSA Antibody (MBS177695) overnight at 4 degree C. Biotinylated goat anti-rabbit IgG was used as secondary antibody and incubated for 30 minutes at 37 degree C. The tissue section was developed using Strepavidin-Biotin-Complex (SABC) with DAB as the chromogen.

Figure 4. Flow Cytometry analysis of Hela cells using anti-ARSA antibody (MBS177695).
Overlay histogram showing Hela cells stained with MBS177695 (Blue line).The cells were blocked with 10% normal goat serum. And then incubated with rabbit anti-ARSA Antibody (MBS177695,1ug/1x10^6 cells) for 30 min at 20 degree C. DyLight?488 conjugated goat anti-rabbit IgG (5-10ug/1x10^6 cells) was used as secondary antibody for 30 minutes at 20 degree C. Isotype control antibody (Green line) was rabbit IgG (1ug/1x106) used under the same conditions. Unlabelled sample (Red line) was also used as a control.

Figure 5. Flow Cytometry analysis of PC-3 cells using anti-ARSA antibody (MBS177695).
Overlay histogram showing PC-3 cells stained with MBS177695 (Blue line).The cells were blocked with 10% normal goat serum. And then incubated with rabbit anti-ARSA Antibody (MBS177695,1ug/1x10^6 cells) for 30 min at 20 degree C. DyLight?488 conjugated goat anti-rabbit IgG (5-10ug/1x10^6 cells) was used as secondary antibody for 30 minutes at 20 degree C. Isotype control antibody (Green line) was rabbit IgG (1ug/1x106) used under the same conditions. Unlabelled sample (Red line) was also used as a control.

44,881 Da

arylsulfatase A

arylsulfatase A

Arylsulfatase A

ASA??[Similar Products]

ARSA_HUMAN

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]

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 *****. 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: EC 3.1.6.8; Hydrolase; 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

1. Fujii T; Kobayashi T; Honke K et al. (1992). "Proteolytic processing of human lysosomal arylsulfatase A". Biochim. Biophys. Acta 1122 (1): 93-8. 2. Kondo R; Wakamatsu N; Yoshino H et al. (1991)."Identification of a mutation in the arylsulfatase A gene of a patient with *****-type metachromatic leukodystrophy". Am. J. Hum. Genet. 48 (5): 971-8. 3. Matzner U, Herbst E, Hedayati KK, Lüllmann-Rauch R, Wessig C, Schr?der S, Eistrup C, M?ller C, Fogh J, Gieselmann V (May 2005). "Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy". Hum. Mol. Genet. 14 (9): 1139-52.

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