MOCS2 Human

Molybdenum Cofactor Synthesis 2 Human Recombinant
Cat. No.
BT25000
Source
E.coli.
Synonyms
Molybdenum Cofactor Synthesis 2, Molybdopterin Synthase Sulfur Carrier Subunit, Molybdenum Cofactor Biosynthesis Protein E, MPT Synthase Large Subunit, Molybdopterin Synthase Catalytic Subunit, Molybdenum Cofactor Synthesis Protein 2 Large Subunit, Molybdenum Cofactor Synthesis Protein 2 Small Subunit, Molybdenum Cofactor Synthesis Protein 2A, Molybd enum Cofactor Synthesis Protein 2B, Sulfur Carrier Protein MOCS2A, MCBPE, MOCO1, MOCO1-A, MOCO1-B, MOCS2A, MOCS2B, MPTS, EC 2.8.1.12.
Appearance
Sterile Filtered colorless solution.
Purity
Greater than 90% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

MOCS2 Human Recombinant produced in E. coli is a single polypeptide chain containing 224 amino acids (1-188) and having a molecular mass of 25.0 kDa.
MOCS2 is fused to a 36 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
Molybdenum Cofactor Synthesis 2 (MOCS2) is a heterotetrameric synthase made up of two small subunits (MOCS2A) and two large subunits (MOCS2B). The MOCS2 gene encodes both the small and large subunits of molybdopterin synthase through overlapping open reading frames. MOCS2 is involved in the second step of the molybdenum cofactor or molybdopterin (MPT) synthesis pathway. In this step, MOCS2 catalyzes the formation of MPT from precursor Z by incorporating a dithiolene functional group.
Description
Recombinant human MOCS2 protein was produced in E. coli. It is a single polypeptide chain containing 224 amino acids (residues 1-188) with a molecular weight of 25.0 kDa. A 36 amino acid His-tag is present at the N-terminus. Purification was achieved using proprietary chromatographic techniques.
Physical Appearance
A sterile, filtered, and colorless solution.
Formulation
The MOCS2 solution has a concentration of 0.25 mg/mL and contains 20 mM Tris-HCl buffer (pH 8.0), 0.15 M NaCl, 1 mM DTT, and 50% glycerol.
Stability
For short-term storage (2-4 weeks), the product can be stored at 4°C. For long-term storage, it is recommended to store the product frozen at -20°C. Adding a carrier protein (0.1% HSA or BSA) is recommended for long-term storage. Avoid repeated freeze-thaw cycles.
Purity
Purity is greater than 90% as determined by SDS-PAGE.
Synonyms
Molybdenum Cofactor Synthesis 2, Molybdopterin Synthase Sulfur Carrier Subunit, Molybdenum Cofactor Biosynthesis Protein E, MPT Synthase Large Subunit, Molybdopterin Synthase Catalytic Subunit, Molybdenum Cofactor Synthesis Protein 2 Large Subunit, Molybdenum Cofactor Synthesis Protein 2 Small Subunit, Molybdenum Cofactor Synthesis Protein 2A, Molybd enum Cofactor Synthesis Protein 2B, Sulfur Carrier Protein MOCS2A, MCBPE, MOCO1, MOCO1-A, MOCO1-B, MOCS2A, MOCS2B, MPTS, EC 2.8.1.12.
Source
E.coli.
Amino Acid Sequence
MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSMSSL EISSSCFSLE TKLPLSPPLV EDSAFEPSRK DMDEVEEKSK DVINFTAEKL SVDEVSQLVI SPLCGAISLF VGTTRNNFEG KKVISLEYEA YLPMAENEVR KICSDIRQKW PVKHIAVFHR LGLVPVSEAS IIIAVSSAHR AASLEAVSYA IDTLKAKVPI WKKEIYEESS TWKGNKECFW ASNS

Product Science Overview

Introduction

Molybdenum Cofactor Synthesis 2 (MOCS2) is a crucial protein involved in the biosynthesis of the molybdenum cofactor (MoCo), which is essential for the activity of various molybdoenzymes in humans. These enzymes play significant roles in metabolic pathways, including the catabolism of sulfur-containing amino acids and purines.

Gene and Protein Structure

The MOCS2 gene is located on chromosome 5 and encodes two subunits of the molybdopterin synthase enzyme: MOCS2A and MOCS2B . These subunits are produced from overlapping open reading frames within the same gene, a unique feature that allows for the efficient synthesis of both components required for molybdopterin biosynthesis .

Function

MOCS2 functions in the second step of MoCo biosynthesis. It catalyzes the conversion of precursor Z into molybdopterin (MPT) by incorporating two sulfur atoms into the precursor to form a dithiolene group . This step is critical for the formation of the active site of molybdoenzymes.

Recombinant MOCS2

Recombinant human MOCS2 is produced using genetic engineering techniques, typically in bacterial systems such as E. coli . The recombinant protein often includes a His-tag to facilitate purification and is used in research to study the function and structure of MOCS2, as well as its role in MoCo biosynthesis .

Clinical Significance

Mutations in the MOCS2 gene can lead to molybdenum cofactor deficiency (MoCD), a rare but severe metabolic disorder characterized by neurological abnormalities and early childhood mortality . MoCD is classified into different types based on the specific gene affected in the biosynthetic pathway, with MOCS2 mutations leading to Type B deficiency .

Research and Applications

Research on MOCS2 and its recombinant forms has provided valuable insights into the mechanisms of MoCo biosynthesis and the pathogenesis of MoCD. Understanding these processes is crucial for developing potential therapies for MoCD and other related disorders .

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