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Adenylosuccinate Lyase Polyclonal Antibody

Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - Adenylosuccinate Lyase Polyclonal Antibody AP58422
    Sample:
    Lane 1: Stomach (Mouse) Lysate at 40 ug
    Lane 2: Spleen (Mouse) Lysate at 40 ug
    Lane 3: Jurkat (Human) Cell Lysate at 30 ug
    Lane 4: HL60 (Human) Cell Lysate at 30 ug
    Lane 5: 293T (Human) Cell Lysate at 30 ug
    Primary: Anti-Adenylosuccinate Lyase (AP58422) at 1/1000 dilution
    Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
    Predicted band size: 55/48 kD
    Observed band size: 50 kD
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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, E
Primary Accession P30566
Reactivity Rat, Pig, Dog, Bovine
Host Rabbit
Clonality Polyclonal
Calculated MW 54889 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human Adenylosuccinate Lyase
Epitope Specificity 185-280/484
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily.
DISEASE Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) . ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions Adenylsuccinate lyase is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. It catalyzes two reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to give aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to give AMP. Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and , in some cases, growth retardation associated with muscle wasting and epilepsy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].
Additional Information
Gene ID 158
Other Names Adenylosuccinate lyase, ADSL, ASL, 4.3.2.2, Adenylosuccinase, ASase, ADSL, AMPS
Target/Specificity Ubiquitously expressed. Both isoforms are produced by all tissues. Isoform 2 is 10-fold less abundant than isoform 1.
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000
Format0.01M TBS(pH7.4) with 1% BSA, 0.09% (W/V) sodium azide and 50% Glyce
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.
Protein Information
Name ADSL
Synonyms AMPS
Function Catalyzes two non-sequential steps in de novo AMP synthesis: converts (S)-2-(5-amino-1-(5-phospho-D-ribosyl)imidazole-4- carboxamido)succinate (SAICAR) to fumarate plus 5-amino-1-(5-phospho-D- ribosyl)imidazole-4-carboxamide, and thereby also contributes to de novo IMP synthesis, and converts succinyladenosine monophosphate (SAMP) to AMP and fumarate.
Tissue Location Ubiquitously expressed. Both isoforms are produced by all tissues. Isoform 2 is 10-fold less abundant than isoform 1
Research Areas

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

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