Aminomethyltransferase; mitochondrial; Glycine cleavage system T protein; GCVT; AMT; GCST

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This AMT antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 19-45 amino acids from the N-terminal region of human AMT.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-AMT antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase; MIM 238300), H protein (a lipoic acid-containing protein; MIM 238330), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899) may be due to a defect in any one of these enzymes.

Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)

WB~~1:1000

Western blot analysis of AMT Antibody (N-term) in HepG2 cell line lysates (35ug/lane). AMT (arrow) was detected using the purified Pab.

Formalin-fixed and paraffin-embedded human hepatocarcinoma reacted with AMT Antibody (N-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.

AMT Antibody (N-term) flow cytometry analysis of HepG2 cells (bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

43946

aminomethyltransferase

aminomethyltransferase, mitochondrial

Aminomethyltransferase, mitochondrial

GCST; GCVT??[Similar Products]

GCST_HUMAN

This gene encodes one of four critical components of the glycine cleavage system. Mutations in this gene have been associated with glycine encephalopathy. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Sep 2011]

AMT: The glycine cleavage system catalyzes the degradation of glycine. Defects in AMT are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvT family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Amino Acid Metabolism - glycine, serine and threonine; Methyltransferase; EC 2.1.2.10; Energy Metabolism - nitrogen; Cofactor and Vitamin Metabolism - one carbon pool by folate; Mitochondrial

Chromosomal Location of Human Ortholog: 3p21.31

Cellular Component: mitochondrion

Molecular Function: transaminase activity; aminomethyltransferase activity

Biological Process: methylation; glycine catabolic process

Disease: Glycine Encephalopathy

Nanao,K., Genomics 19 (1), 27-30 (1994)

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