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ARSA, Polyclonal Antibody

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產品名稱: ARSA, Polyclonal Antibody
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ARSA, Polyclonal Antibody


ARSA, Polyclonal Antibody  的詳細介紹
Product Name

ARSA, Polyclonal Antibody

Full Product Name

ARSA Antibody

Product Synonym Names
MLD
Product Gene Name

anti-ARSA antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
250100
3D Structure
ModBase 3D Structure for P15289
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen affinity purification
Immunogen
Fusion protein of ARSA
Calculated Molecular Weight: 54kd
Observed Molecular Weight: 56kd
Buffer
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-68914 / sc-79848 / sc-79845
Preparation and Storage
Store at -20 degree C. Avoid freeze / thaw cycles
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
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.
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Applications Tested/Suitable for anti-ARSA antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-ARSA antibody
WB: 1:500-1:5000
IHC: 1:20-1:200

Testing Data of anti-ARSA antibody
anti-ARSA antibody Testing Data image
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NCBI/Uniprot data below describe general gene information for ARSA. It may not necessarily be applicable to this product.
NCBI GI #
47678297
NCBI GeneID
410
NCBI Accession #
CAG30269.1 [Other Products]
UniProt Primary Accession #
P15289 [Other Products]
UniProt Secondary Accession #
Q6ICI5; Q96CJ0; B2RCA6; B7XD04; F8WCC8[Other Products]
UniProt Related Accession #
P15289[Other Products]
Molecular Weight
44,881 Da
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NCBI Official Full Name
ARSA
NCBI Official Synonym Full Names
arylsulfatase A
NCBI Official Symbol
ARSA??[Similar Products]
NCBI Official Synonym Symbols
MLD
??[Similar Products]
NCBI Protein Information
arylsulfatase A; ASA; cerebroside-sulfatase
UniProt Protein Name
Arylsulfatase A
UniProt Synonym Protein Names
Cerebroside-sulfataseCleaved into the following 2 chains:Arylsulfatase A component B; Arylsulfatase A component C
Protein Family
Arylsulfatase
UniProt Gene Name
ARSA??[Similar Products]
UniProt Synonym Gene Names
ASA??[Similar Products]
UniProt Entry Name
ARSA_HUMAN
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NCBI Summary for ARSA
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]
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UniProt Comments for ARSA
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: Lipid Metabolism - sphingolipid; EC 3.1.6.8; Hydrolase

Chromosomal Location of Human Ortholog: 22q13.33

Cellular Component: lysosomal lumen; extracellular space; extrinsic to external side of plasma membrane; lysosome; endoplasmic reticulum lumen; integral to membrane; acrosome; endosome

Molecular Function: arylsulfatase activity; sulfuric ester hydrolase activity; calcium ion binding; cerebroside-sulfatase activity

Biological Process: sphingolipid metabolic process; cellular protein metabolic process; response to ethanol; central nervous system development; response to estrogen stimulus; binding of sperm to zona pellucida; response to methylmercury; autophagy; glycosphingolipid metabolic process; post-translational protein modification; response to nutrient; response to pH

Disease: Metachromatic Leukodystrophy
Research Articles on ARSA
1. Sixteen novel mutations that cause metachromatic leukodystrophy have been identified in the arylsulfatase A gene.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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