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Image Unavailable-BOSSBS-12515R-FITC

Anti-ASL Rabbit Polyclonal Antibody (FITC (Fluorescein Isothiocyanate))

Catalog Number: (BOSSBS-12515R-FITC)
Supplier: Bioss
Description: ASL is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
UOM: 1 * 100 µl
Image Unavailable-SIAL03761-500G

Fumaric acid, Sigma-Aldrich®

Catalog Number: (SIAL03761-500G)
Supplier: Merck
Description: Fumaric acid, Sigma-Aldrich®
UOM: 1 * 500 g

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Image Unavailable-SIALF1506-500G

Disodium fumarate, Sigma-Aldrich®

Supplier: Merck
Description: Disodium fumarate, Sigma-Aldrich®

Image Unavailable-SIALW248800-1KG-K

Fumaric acid, Sigma-Aldrich®

Supplier: Merck
Description: Fumaric acid, Sigma-Aldrich®

Image Unavailable-8.41773.0025

3-Aminopropionitrile fumarate

Catalog Number: (8.41773.0025)
Supplier: Merck
Description: 3-Aminopropionitrile fumarate
UOM: 1 * 25 g
Image Unavailable-ICNA0219144990

Succinic acid ≥99%, powder ACS

Supplier: MP Biomedicals
Description: Succinic acid is a dicarboxylic acid that occurs in nature in such organisms as fungi and lichens. In eukaryotes, succiniate, the anion of succinic acid, is an intermediate in the citric acid cycle, being formed from succinyl CoA and being converted to fumarate. Bacteria and plants can produce succinate from acetate or acetyl CoA, in the glyoxylate cycle.

Image Unavailable-APOSBIK4000-250MG

Ketotifen fumarate

Supplier: Apollo Scientific
Description: Ketotifen fumarate

Image Unavailable-B25440.14

3-Aminopropionitrile fumarate 98

Supplier: Thermo Fisher Scientific
Description: 3-Aminopropionitrile fumarate 98

SDS Certificates

Product Image-ACRO104470250

3-Aminopropionitrile fumarate 99+%

Supplier: Thermo Fisher Scientific
Description: 3-Aminopropionitrile fumarate 99+%

SDS

Image Unavailable-ENZOBMLNH1060001

Ketotifen fumarate ≥99%

Catalog Number: (ENZOBMLNH1060001)
Supplier: ENZO LIFE SCIENCES
Description: Histamine receptor ligand
UOM: 1 * 1 g

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Image Unavailable-APOSBIT0157-1G

Tiamulin fumarate

Supplier: Apollo Scientific
Description: Tiamulin fumarate

Image Unavailable-PRSI91-204

Human recombinant fumarase (from E. coli)

Catalog Number: (PRSI91-204)
Supplier: ProSci Inc.
Description: Fumarase is an enzyme that catalyze the reversible hydration/dehydration of fumarate to S-malate and is involved in the tricarboxylic acid or Krebs cycle. Fumarase exists in both form, cytosolic formand N-terminal extend mitochondrial form. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension is the same form as in the cytoplasm. Fumarase is thought to act as a tumour suppressor, which deficiency can lead to progressive encephalopathy, cerebral atrophy and development delay.
UOM: 1 * 50 µG
Product Image-ACRO465422500

Clemastine fumarate

Supplier: Thermo Fisher Scientific
Description: Clemastine fumarate

Product Image-PRSI29-614

Anti-ASL Rabbit Polyclonal Antibody

Catalog Number: (PRSI29-614)
Supplier: ProSci Inc.
Description: ASL is a member of the lyase 1 family. The protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in its gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency.This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described.
UOM: 1 * 50 µG
Image Unavailable-APOSPC3906-5G

Bis(perfluoroalkylethyl) fumarate

Catalog Number: (APOSPC3906-5G)
Supplier: Apollo Scientific
Description: Bis(perfluoroalkylethyl) fumarate
UOM: 1 * 5 g
Image Unavailable-ACRO465912500

Bedaquiline fumarate

Supplier: Thermo Fisher Scientific
Description: Bedaquiline fumarate

Certificates

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