You Searched For: Porphobilinogen+monohydrate

Catalog Number: (PRSI91-696)

Supplier: ProSci Inc.

Description: Porphobilinogen Deaminase (HMBS) is a member of the HMBS family. PBGD is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. HMBS is involved in the production of heme, which is important for all of the body's organs, although it is most abundant in the blood, bone marrow, and liver. In addition, Heme is an essential component of iron-containing proteins called hemoproteins, including hemoglobin. Defects in PBGD are the cause of acute intermittent porphyria.

UOM: 1 * 50 µG

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Catalog Number: (PRSI30-974)

Supplier: ProSci Inc.

Description: HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.

UOM: 1 * 50 µG

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Catalog Number: (PRSI26-873)

Supplier: ProSci Inc.

Description: HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.

UOM: 1 * 50 µG

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Catalog Number: (PRSI29-613)

Supplier: ProSci Inc.

Description: The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria.The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternatively spliced transcript variants encoding different isoforms have been identified.

UOM: 1 * 50 µG

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Catalog Number: (PRSI29-612)

Supplier: ProSci Inc.

Description: The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria.The ALAD enzyme is composed of 8 identical subunits and catalyzes the condensation of 2 molecules of delta-aminolevulinate to form porphobilinogen (a precursor of heme, cytochromes and other hemoproteins). ALAD catalyzes the second step in the porphyrin and heme biosynthetic pathway; zinc is essential for enzymatic activity. ALAD enzymatic activity is inhibited by lead and a defect in the ALAD structural gene can cause increased sensitivity to lead poisoning and acute hepatic porphyria. Alternatively spliced transcript variants encoding different isoforms have been identified.

UOM: 1 * 50 µG

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Catalog Number: (PRSI30-297)

Supplier: ProSci Inc.

Description: The developmentally regulated expression of the globin genes depends on upstream regulatory elements termed locus control regions (LCRs). LCRs are associated with powerful enhancer activity that is mediated by the transcription factor NFE2. NFE2 recognition sites are also present in the gene promoters of 2 heme biosynthetic enzymes, PBGD and FECH. NFE2 DNA-binding activity consists of a heterodimer containing an 18-kD Maf protein (MafF, MafG, or MafK) and p45. Both subunits are members of the activator protein-1 superfamily of bZIP proteins. Maf homodimers suppress transcription at NFE2 sites.The developmentally regulated expression of the globin genes depends on upstream regulatory elements termed locus control regions (LCRs). LCRs are associated with powerful enhancer activity that is mediated by the transcription factor NFE2 (nuclear factor erythroid-2). NFE2 recognition sites are also present in the gene promoters of 2 heme biosynthetic enzymes, porphobilinogen deaminase (PBGD; MIM 176000) and ferrochelatase (FECH; MIM 177000). NFE2 DNA-binding activity consists of a heterodimer containing an 18-kD Maf protein (MafF, MafG (MIM 602020), or MafK) and p45 (MIM 601490). Both subunits are members of the activator protein-1 superfamily of basic leucine zipper (bZIP) proteins (see MIM 165160). Maf homodimers suppress transcription at NFE2 sites.[supplied by OMIM].

UOM: 1 * 100 µG

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Supplier: Avantor

Description: D-Lactose monohydrate, powder NF, multi-compendial, J.T.Baker®

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Supplier: SIGMA ALDRICH MICROSCOPY

Description: Reagent for the preparation of heavy liquid, for sink-float analysis.

Supplier: Avantor

Description: L(+)-Asparagine monohydrate NF, multi-compendial, J.T.Baker®

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Catalog Number: (29246.298)

Supplier: VWR Chemicals

Description: Zinc sulphate monohydrate USP

UOM: 1 * 1 kg

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Supplier: VWR Chemicals

Description: D-(+)-Glucose monohydrate Biochemistry

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Supplier: VWR Chemicals

Description: 1,10-Phenanthroline monohydrate 99% for synthesis

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Supplier: Avantor

Description: D-Lactose monohydrate, powder NF, J.T.Baker®

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Supplier: Avantor

Description: D-Lactose monohydrate, powder, BAKER ANALYZED® ACS, J.T.Baker®

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Catalog Number: (0643-1KG)

Supplier: VWR Chemicals

Description: D-(+)-Glucose monohydrate ≥99.5% for biotechnology

UOM: 1 * 1 kg

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Catalog Number: (0248-500G)

Supplier: VWR Chemicals

Description: Manganese(II) sulphate monohydrate, Reagent Grade

UOM: 1 * 500 g

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