You Searched For: Disodium+fumarate

Catalog Number: (76714-248)

Supplier: AFG Bioscience

Description: Human Fumarate hydratase, Mitochondrial(FH) ELISA Kit

Catalog Number: (103636-432)

Supplier: Sino Biological

Description: This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human Fumarate Hydratase / FH (rh Fumarate Hydratase / FH; Catalog#12115-H08E; P07954-1; Ala 45-Lys 510). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.

Supplier: ZING Enterprises

Description: The sign is available in 1 size, 14 x 10", and two eco-friendly formats.

Catalog Number: (76085-374)

Supplier: Bioss

Description: Defects in FH are the cause of fumarase deficiency (FHD)also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia.

Supplier: MilliporeSigma

SDS Certificates

Catalog Number: (10435-758)

Supplier: Bioss

Description: 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].

Catalog Number: (10795-280)

Supplier: Genetex

Description: Rabbit Polyclonal antibody to Fumarate hydratase

Catalog Number: (EM8.04015.0100)

Supplier: MilliporeSigma

Description: Furfuryl alcohol for synthesis

SDS Certificates

Catalog Number: (10082-132)

Supplier: Proteintech

Description: FH(fumarate hydratase) belongs to the class-II fumarase/aspartase family. It is an enzymatic component of the tricarboxylic acid cycle, catalyzing the conversion of fumarate to malate. FH also acts a tumor suppressor functions of FH in sporadic kidney cancer. This protein has 2 isoforms produced by alternative initiation.

Catalog Number: (10435-772)

Supplier: Bioss

Description: 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].

Catalog Number: (10108-306)

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.

Catalog Number: (10435-776)

Supplier: Bioss

Description: 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].

Catalog Number: (10435-774)

Supplier: Bioss

Description: 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].

Catalog Number: (103622-976)

Supplier: Sino Biological

Description: A DNA sequence encoding the mature form of human FH (P07954-1) (Ala 45-Lys 510) was fused with a polyhistidine tag at the C-terminus and an initial Met at the N-terminus.

Catalog Number: (10435-770)

Supplier: Bioss

Description: 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].

Catalog Number: (103636-436)

Supplier: Sino Biological

Description: Produced in rabbits immunized with purified, recombinant Human Fumarate Hydratase (rh FH; Catalog#12115-H08E; P07954-1; Ala45-Lys510). Total IgG was purified by Protein A affinity chromatography.