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Iduronate 2 sulfatase Rabbit pAb

Iduronate 2 sulfatase Rabbit pAb

     
  • 1 - Iduronate 2 sulfatase Rabbit pAb AP56033
    Sample:
    Hela Cell(Human)Lysate at 30 ug
    Primary: Anti- Iduronate 2 sulfatase (AP56033)at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 47kD
    Observed band size: 62kD
  • 14 - Iduronate 2 sulfatase Rabbit pAb AP56033
    Paraformaldehyde-fixed, paraffin embedded (Human kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Iduronate 2 sulfatase) Polyclonal Antibody, Unconjugated (AP56033) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF
Primary Accession P22304
Reactivity Human
Predicted Rat, Mouse, Dog, Sheep
Host Rabbit
Clonality Polyclonal
Calculated MW 61873 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human Iduronate 2 sulfatase
Epitope Specificity 101-200/550
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Lysosome.
DISEASE Mucopolysaccharidosis 2 (MPS2) [MIM:309900]: An X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Note=The disease is caused by mutations affecting the gene represented in this entry.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013]
Additional Information
Gene ID 3423
Other Names Iduronate 2-sulfatase, 3.1.6.13, Alpha-L-iduronate sulfate sulfatase, Idursulfase, Iduronate 2-sulfatase 42 kDa chain, Iduronate 2-sulfatase 14 kDa chain, IDS, SIDS
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name IDS
Synonyms SIDS
Function Lysosomal enzyme involved in the degradation pathway of dermatan sulfate and heparan sulfate.
Cellular Location Lysosome.
Tissue Location Liver, kidney, lung, and placenta.
Research Areas

BACKGROUND

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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