Western Blot (WB)
(Western blot analysis of ADSL expression in HepG2 (A), Jurkat (B), mouse heart (C), rat brain (D) whole cell lysates.)
Rabbit ADSL Polyclonal Antibody | anti-ADSL antibody
Anti-ADSL Antibody
Western Blot (WB)
(Western blot analysis of ADSL expression in HepG2 (A), Jurkat (B), mouse heart (C), rat brain (D) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of ADSL expression in HepG2 (A), Jurkat (B), mouse heart (C), rat brain (D) whole cell lysates.)
Immunofluorescence (IF)
(Immunofluorescent analysis of ADSL staining in Hela cells. Formalin-fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 5-10 minutes and blocked with 3% BSA-PBS for 30 minutes at room temperature. Cells were probed with the primary antibody in 3% BSA-PBS and incubated overnight at 4 °C in a humidified chamber. Cells were washed with PBST and incubated with a DyLight 594-conjugated secondary antibody (red) in PBS at room temperature in the dark.)
Immunofluorescence (IF)
(Immunofluorescent analysis of ADSL staining in Hela cells. Formalin-fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 5-10 minutes and blocked with 3% BSA-PBS for 30 minutes at room temperature. Cells were probed with the primary antibody in 3% BSA-PBS and incubated overnight at 4 °C in a humidified chamber. Cells were washed with PBST and incubated with a DyLight 594-conjugated secondary antibody (red) in PBS at room temperature in the dark.)
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the conversion of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in this gene are associated with adenylosuccinase deficiency (ADSLD), a disorder marked with psychomotor retardation, epilepsy or autistic features. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Dec 2015]
Uniprot Description
ADSL: 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. Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Nucleotide Metabolism - purine; EC 4.3.2.2; Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate
Chromosomal Location of Human Ortholog: 22q13.2
Cellular Component: cytosol
Molecular Function: adenylosuccinate lyase activity
Biological Process: 'de novo' IMP biosynthetic process; AMP biosynthetic process; protein tetramerization; purine nucleotide biosynthetic process; purine ribonucleoside monophosphate biosynthetic process
Disease: Adenylosuccinase Deficiency
Research Articles on ADSL
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Product Notes
The ADSL adsl (Catalog #AAA8245655) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-ADSL Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's ADSL can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC). WB: 1/500 - 1/2000; IF/ICC: 1/10 - 1/100. Researchers should empirically determine the suitability of the ADSL adsl for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ADSL, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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