You Searched For: Dimethyl+fumarate

1,900 results were found

Refine Results Sort by

Sort Results

List View Easy View

Compare(0) Clear

Rate These Search Results

Anti-FAH Rabbit Polyclonal Antibody

Catalog Number: (10086-770)

Supplier: Proteintech

Description: FAH is also named as FAA(fumarylacetoacetase), the last enzyme in tyrosine degradation, functionally important in the liver and kidney. FAH is a metabolic enzyme catalyzing the last step of tyrosine and phenylalanine catabolism: the hydrolysis of fumarylacetoacetate into acetoacetate and fumarate. In humans, deficiency of this activity is associated with the metabolic disease hereditary tyrosinaemia type I, which is also known as hepatorenal tyrosinaemia.

Retrieving Each

Anti-ASL Rabbit Polyclonal Antibody

Catalog Number: (10108-304)

Supplier: Prosci

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.

Retrieving Each

Anti-ADSL Rabbit Polyclonal Antibody

Catalog Number: (10082-556)

Supplier: Proteintech

Description: ADSL(adenylosuccinate lyase) is also named as AMPS, ASase, ASL and belongs to the lyase 1 family. It is an enzyme involved in 2 pathways of purine nucleotide metabolism and catalyzes cleavage of succinyl groups to yield fumarate. Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). In humans, mutations in ADSL lead to an inborn error of metabolism originally characterized by developmental delay, often with autistic features..The ADSL enzymatic activity is reduced in lymphocytes and red blood cells of the patient with severe psychomotor retardation. It has 2 isoforms produced by alternative splicing.

Retrieving Each

Anti-HGD Rabbit Polyclonal Antibody (ALEXA FLUOR® 680)

Catalog Number: (76099-998)

Supplier: Bioss

Description: HGD is a 445 amino acid protein that belongs to the homogentisate dioxygenase family and is involved in the pathway of amino acid degradation. Expressed at high levels in kidney, colon, liver, prostate and small intestine, HGD uses iron as a cofactor to catalyze the oxygen-dependent conversion of homogentisate to 4-maleylacetoacetate, a reaction that is the fourth step in the creation of L-phenylalanine from fumarate and acetoacetic acid. Defects in the gene encoding HGD are the cause of alkaptonuria (AKU), an autosomal recessive disorder that is characterized by urine that turns dark on standing and alkalinization, black ochronotic pigmentation of cartilage and collagenous tissues and spine arthritis.

Retrieving Each

Human Recombinant 4HPPD (from E. coli)

Catalog Number: (75790-626)

Supplier: Prosci

Description: 4-Hydroxyphenylpyruvate Dioxygenase (4HPPD) belongs to the 4HPPD family. 4HPPD is a key enzyme in the degradation of tyrosine, which catalyzes the second reaction in the catabolism of tyrosine the conversation of 4-hydroxyphenylpyruvate to homogentisate. 4HPPD exists in homodimer forms, which uses zinc as a cofactor to catalyze the third step in the conversion of L-phenylalanine to fumarate and acetoacetic acid. When the active 4HPPD enzyme concentration is low in the human body, it results in high levels of tyrosine concentration in the blood, which can cause mild mental retardation at birth, and degradation in vision as a patient grows older.

Retrieving Each

Anti-HGD Rabbit Polyclonal Antibody (ALEXA FLUOR® 750)

Catalog Number: (76100-000)

Supplier: Bioss

Retrieving Each

Anti-Fumarase/FH Goat Polyclonal Antibody

Catalog Number: (CAPIPA5-18745)

Supplier: Thermo Scientific

Description: This antibody is predicted to react with canine, mouse and rat based on sequence homology. The protein encoded by this gene is an enzymatic component of the tricarboxylic acid cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.