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Argininosuccinic Aciduria: Disease Bioinformatics

Research of Argininosuccinic Aciduria has been linked to Hyperammonemia, Ornithine Carbamoyltransferase Deficiency, Inborn Errors Of Metabolism, Citrullinemia, Urea Cycle Disorders, Inborn. The study of Argininosuccinic Aciduria has been mentioned in research publications which can be found using our bioinformatics tool below. Researched pathways related to Argininosuccinic Aciduria include Urea Cycle, Excretion, Pathogenesis, Transport, Coagulation. These pathways complement our catalog of research reagents for the study of Argininosuccinic Aciduria including antibodies and ELISA kits against AMINO-ACID N-ACETYLTRANSFERASE, CARBAMYL PHOSPHATE SYNTHETASE I, HISTIDINEMIA, CPS, L-GULONOLACTONE OXIDASE.

Top Research Reagents

We have 1902 products for the study of Argininosuccinic Aciduria that can be applied to Flow Cytometry, Immunocytochemistry/ Immunofluorescence, Immunohistochemistry, Western Blot from our catalog of antibodies and ELISA kits.

iNOS Antibody - BSA Free
NB300-605
Western Blot: iNOS Antibody [NB300-605] - Analysis of iNOS was performed by loading 20 ug of RAW264 whole cell lysate untreated (left lane) or stimulated with LPS at 1 ug/mL for 16 hours (right lane) and 10 uL of PageRuler Plus Prestained Protein Ladder onto a 4-20% Tris-Glycine polyacrylamide gel. Proteins were transferred to a nitrocellulose membrane and blocked with 5% Milk in TBST for at least 1 hour. The membrane was probed with an iNOS Rabbit polyclonal antibody at a dilution of 1:1000 overnight at 4C on a rocking platform, washed in TBST, and probed with a Goat anti-Rabbit IgG (H+L) Secondary Antibody, HRP conjugate at a dilution of 1:1000 for 1 hour. Chemiluminescent detection was performed using SuperSignal West Pico.Immunohistochemistry-Paraffin: iNOS Antibody [NB300-605] - Immunohistochemistry was performed on normal deparaffinized human Lung tissue.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, Flow, IB

     3 Reviews

141 Publications
Arylsulfatase A/ARSA Antibody
NBP1-00154
Western Blot: Arylsulfatase A/ARSA Antibody [NBP1-00154] - Western blot analysis of Mouse testes lysate (A) + peptide (B) with antibody at 1 ug/mL and Rat testes lysate (C) + peptide (D) with antibody at 0.3 ug/mL, 35ug protein in RIPA buffer. Detected by chemiluminescence.Immunocytochemistry/Immunofluorescence: Arylsulfatase A/ARSA Antibody [NBP1-00154] - Immunofluorescence analysis of paraformaldehyde fixed HeLa cells, permeabilized with 0.15% Triton. Primary incubation 1hr (10 ug/mL) followed by Alexa Fluor 488 secondary antibody (2 ug/mL), showing Golgi apparatus staining. The nuclear stain is DAPI (blue). Negative control: Unimmunized goat IgG (10 ug/mL) followed by Alexa Fluor 488 secondary antibody (2 ug/mL).

Goat Polyclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

1 Publication
DEGS1 Antibody (PSH02-54)
NBP3-32598
Western Blot: DEGS1 Antibody (PSH02-54) [NBP3-32598] - Western blot analysis of DEGS1 on different lysates with Rabbit anti-DEGS1 antibody (NBP3-32598) at 1/1,000 dilution.<br/><br/> Lane 1: A549 cell lysate<br/> Lane 2: 293T cell lysate<br/> Lane 3: HeLa cell lysate<br/> Lane 4: PC-3M cell lysate<br/> Lane 5: NIH/3T3 cell lysate<br/><br/> Lysates/proteins at 20 ug/Lane.<br/><br/> Predicted band size: 38 kDa<br/> Observed band size: 34 kDa<br/><br/> Exposure time: 5 minutes;<br/><br/> 4-20% SDS-PAGE gel.<br/><br/> Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (NBP3-32598) at 1/1,000 dilution was used in 5% NFDM/TBST at 4 overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody at 1/50,000 dilution was used for 1 hour at room temperature.Immunohistochemistry: DEGS1 Antibody (PSH02-54) [NBP3-32598] - Immunofluorescence analysis of paraffin-embedded human skin tissue labeling DEGS1 with Rabbit anti-DEGS1 antibody (NBP3-32598) at 1/200 dilution.<br/><br/> The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 10% negative goat serum for 1 hour at room temperature, washed with PBS, and then probed with the primary antibody (NBP3-32598, green) at 1/200 dilution overnight at 4 ℃, washed with PBS. Goat Anti-Rabbit IgG H&L (iFluor™ 488) was used as the secondary antibody at 1/1,000 dilution. Nuclei were counterstained with DAPI (blue).

Rabbit Monoclonal
Species Human, Mouse, Rat
Applications WB, IHC, IHC-P

Fumarase Antibody - BSA Free
NBP1-31336
Western Blot: Fumarase Antibody [NBP1-31336] - Non-transfected (-) and transfected (+) 293T whole cell extracts (30 ug) were separated by 10% SDS-PAGE, and the membrane was blotted with Fumarate hydratase antibody. HRP-conjugated anti-rabbit IgG antibody was used to detect the primary antibody.Western Blot: Fumarase Antibody [NBP1-31336] - Representative western blots showing protein expression of fumarase and key enzymes in OXPHOS. VDAC was used as a loading control (n = 3). Image collected and cropped by CiteAb from the following publication (https://www.nature.com/articles/srep22788) licensed under a CC-BY license.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

     1 Review

1 Publication
PEPD Antibody - BSA Free
NBP1-86072
Immunohistochemistry-Paraffin: PEPD Antibody [NBP1-86072] - Analysis in human kidney and skeletal muscle tissues. Corresponding PEPD RNA-seq data are presented for the same tissues.Western Blot: PEPD Antibody [NBP1-86072] - Lane 1: Marker [kDa] 230, 130, 95, 72, 56, 36, 28, 17, 11. Lane 2: Human cell line RT-4. Lane 3: Human cell line U-251MG sp. Lane 4: Human plasma (IgG/HSA depleted). Lane 5: Human liver tissue. Lane 6: Human tonsil tissue

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ICC/IF, IHC

Ornithine Carbamoyltransferase Antibody - BSA Free
NBP1-87408
Immunohistochemistry-Paraffin: Ornithine Carbamoyltransferase Antibody [NBP1-87408] - Staining in human liver and kidney tissues . Corresponding OTC RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Ornithine Carbamoyltransferase Antibody [NBP1-87408] - Staining of human cerebral cortex, kidney, liver and small intestine using Anti-OTC antibody NBP1-87408 (A) shows similar protein distribution across tissues to independent antibody NBP1-88121 (B).

Rabbit Polyclonal
Species Human, Mouse
Applications WB, Simple Western, ICC/IF

5 Publications
Argininosuccinate Lyase Antibody - BSA Free
NBP1-87462
Immunohistochemistry-Paraffin: Argininosuccinate Lyase Antibody [NBP1-87462] - Staining in human liver and skeletal muscle tissues using NBP1-87462 antibody. Corresponding ASL RNA-seq data are presented for the same tissues.Immunohistochemistry-Paraffin: Argininosuccinate Lyase Antibody [NBP1-87462] - Staining of human kidney shows moderate to strong cytoplasmic positivity in cells in tubules.

Rabbit Polyclonal
Species Human, Canine
Applications WB, IHC, IHC-P

2 Publications
Argininosuccinate Synthase Antibody - BSA Free
NBP1-88868
Western Blot: Argininosuccinate Synthase Antibody [NBP1-88868] - ASS1 was successfully silenced in H4-II-E-C3 cells. Image from verified customer review.Immunocytochemistry/Immunofluorescence: Argininosuccinate Synthase Antibody [NBP1-88868] - Staining of human cell line A-431 shows localization to nucleoplasm & cytosol. Antibody staining is shown in green.

Rabbit Polyclonal
Species Human
Applications WB, ICC/IF, IHC

     1 Review

Adenylosuccinate Lyase Antibody (OTI2D10)
NBP2-03107
Western Blot: Adenylosuccinate Lyase Antibody (OTI2D10) [NBP2-03107] - Blot showing the effect of hypoxia on the protein expression levels of the purine biosynthetic enzymes. HIF-1 alpha is stabilized in hypoxia as expected, and no significant increase in the purine enzymes was detected between normoxic (21% oxygen) and hypoxic (1% oxygen) growth conditions. The positions of molecular markers surrounding each band of interest are shown for each blot. ADSL (NBP2-03107), ATIC (NBP2-01941), FGAMS (NBP1-84691), GART (H00002618-M01), HIF-1a (NB100-449), PAICS (NBP2-02817), PPAT (NBP2-02056). Image collected and cropped by CiteAb from the following publication (//pubmed.ncbi.nlm.nih.gov/32439803/) licensed under a CC-BY license.Immunohistochemistry-Paraffin: Adenylosuccinate Lyase Antibody (OTI2D10) [NBP2-03107] - Staining of paraffin-embedded Human lymphoma tissue using anti-Adenylosuccinate Lyase mouse monoclonal antibody.

Mouse Monoclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

2 Publications
Prolactin Antibody [Unconjugated]
AF1445
Western blot shows lysates of mouse pituitary tissue and rat pituitary tissue. PVDF membrane was probed with 0.25 µg/mL of Goat Anti-Mouse/Rat Prolactin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF1445) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (<a class=Recombinant Mouse Prolactin (<a class=

Goat Polyclonal
Species Mouse, Rat
Applications WB, Simple Western, IHC

7 Publications
GM-CSF [Unconjugated]
7954-GM/CF
Measured in a cell proliferation assay using TF-1 human erythroleukemic cells. The ED<sub>50</sub> for this effect is 6-30 pg/mL.


Species Human
Applications BA

3 Publications
LAMC2 Antibody (CL2980) - BSA Free
NBP2-42388
Immunohistochemistry-Paraffin: LAMC2 Antibody (CL2980) [NBP2-42388] - Staining in human fallopian tube and liver tissues. Corresponding LAMC2 RNA-seq data are presented for the same tissues.Western Blot: LAMC2 Antibody (CL2980) [NBP2-42388] - Analysis in A-431 cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-LAMC2 antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.

Mouse Monoclonal
Species Human
Applications WB, ICC/IF, IHC

4 Publications
Lysozyme Antibody - BSA Free
NBP2-61118
Immunocytochemistry/Immunofluorescence: Lysozyme Antibody [NBP2-61118] - NIH3T3 cells were fixed and permeabilized for 10 minutes using -20C MeOH. The cells were incubated with anti-Lysozyme NBP2-61118 at 2 ug/ml overnight at 4C and detected with an anti-rabbit Dylight 488 (Green) at a 1:1000 dilution for 60 minutes. Nuclei were counterstained with DAPI (Blue). Cells were imaged using a 100X objective and digitally deconvolved.Western Blot: Lysozyme Antibody [NBP2-61118] - Total protein from human cell lines THP-1 and HepG2, human stomach and kidney as well as mouse kidney was separated on a 4-20% gel by SDS-PAGE, transferred to 0.2 um PVDF membrane and blocked in 5% non-fat milk in TBST. The membrane was probed with 2.0 ug/ml anti-Lysozyme in 5% non-fat milk in TBST and detected with an anti-rabbit HRP secondary antibody using chemiluminescence.

Rabbit Polyclonal
Species Human, Mouse
Applications WB, Simple Western, Flow

7 Publications
nNOS Antibody
NB100-858
Immunocytochemistry/Immunofluorescence: nNOS Antibody [NB100-858] - Action potentials were evoked with depolarizing current pulses. (A) Neuron from colonic specimen of non-treated patient fired 1 action potential in response to a depolarizing current. (A') Intracellular injection of carboxyfluorescein during recording confirmed Dogiel type I, uniaxonal morphology. (AImmunohistochemistry-Paraffin: nNOS Antibody [NB100-858] - Staining of paraffin embedded Human Cortex. Steamed antigen retrieval with citrate buffer pH 6, AP-staining. Antibody at 2.5 ug/mL.

Goat Polyclonal
Species Human, Mouse, Rat
Applications WB, Flow, ICC/IF

     1 Review

15 Publications
NAGS Antibody - Azide and BSA Free
NBP2-93763
Western Blot: NAGS Antibody [NBP2-93763] - Analysis of extracts of various cell lines, using NAGS at 1:3000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit .Exposure time: 90s.

Rabbit Polyclonal
Species Human, Mouse, Rat
Applications WB, ELISA


Related Genes

Argininosuccinic Aciduria has been researched against:

Related Pathways

Argininosuccinic Aciduria has been linked to:

Related Diseases

Argininosuccinic Aciduria has been studied in relation to diseases such as:

Related PTMs

Argininosuccinic Aciduria has been studied in relation to posttranslational modifications (PTMs) including:

Alternate Names

Argininosuccinic Aciduria is also known as argininosuccinic aciduria, argininosuccinate lyase deficiency, asl deficiency, arginosuccinase deficiency, inborn error of urea synthesis, arginino succinic type, deficiency of argininosuccinate lyase (disorder), argininosuccinate lyase deficiency (disorder), urea cycle disorder, arginino succinase type, argininosuccinyl-coa lyase deficiency, arginino succinase deficiency, argininosuccinate acidemia, argininosuccinic acidemia, aciduria argininosuccinic, argininosuccinicaciduria, deficiency of lyase, malnutrition, acidemia, aciduria, asa.