GCSH antibody

Principal name

GCSH antibody

Alternative names for GCSH antibody

Glycine cleavage system H protein

SwissProt ID

P20821 (Bovin), P23434 (Human), Q5I0P2 (Rat), Q91WK5 (Mouse)

Gene ID

2653 (GCSH)

Available reactivities

Hu (Human), Ms (Mouse), Rt (Rat)

Available hosts

Mouse, Rabbit

Available applications

Enzyme Immunoassay (E), Western blot / Immunoblot (WB), Immunocytochemistry/Immunofluorescence (ICC/IF), Immunoprecipitation (IP), Paraffin Sections (P)

Background of GCSH antibody

GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome.

General readings

Kure S., et al. (2001) J Hum Genet. 46(7):378-84.Kovata H., et al. (1991) Am J Hum Genet. 48(2):351-61.

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Primary Antibodies

Catalog No. Host Iso. Clone Pres. React. Applications  

GCSH antibody

GCSH Mouse Serum Hu E, WB
50 µl / €290.00
  Abnova Taiwan Corp.

GCSH antibody

Detection limit for recombinant GST tagged GCSH is approximately 0.1ng/ml as a capture antibody. Mouse IgG1 3D8-A12 Purified Hu E, WB
0.1 mg / €370.00
  Abnova Taiwan Corp.

GCSH antibody

Detection limit for recombinant GST tagged GCSH is approximately 0.1ng/ml as a capture antibody. Mouse IgG1 M2 Purified Hu E, WB
0.1 mg / €370.00
  Abnova Taiwan Corp.

GCSH antibody

Immunohistochemistry-Paraffin: GCSH Antibody [NBP1-85842] - Immunohistochemical staining of human kidney shows strong granular cytoplasmic positivity in cells in tubules.. Rabbit IgG Aff - Purified Hu P, WB
0.1 ml / €500.00
  Novus Biologicals Inc.

Proteins & Growth Factors

Catalog No. Species Pres. Purity Source  

GCSH

GCSH Human > 80 %
Preparation: Recombint protein was captured through anti-DDK affinity column followed by conventiol chromatography steps.
Purity Detail: > 80% as determined by SDS-PAGE and Coomassie blue staining.
HEK293 cells
20 µg / €680.00
  OriGene Technologies, Inc.

GCSH (49-173, His-tag)

GCSH Human Purified > 95 % by SDS - PAGE E. coli
0.5 mg / €730.00
  Acris Antibodies GmbH

GCSH (49-173, His-tag)

GCSH Human Purified > 95 % by SDS - PAGE E. coli
0.1 mg / €300.00
  Acris Antibodies GmbH

GCSH

GCSH Human 12.5% SDS-PAGE Stained with Coomassie Blue. in vitro transl.
10 µg / €380.00
  Abnova Taiwan Corp.

GCSH

GCSH Human 12.5% SDS-PAGE Stained with Coomassie Blue. in vitro transl.
25 µg / €510.00
  Abnova Taiwan Corp.

Lysates

Catalog No.    

GCSH overexpression lysate

GCSH overexpression lysate  
0.1 mg / €280.00
  OriGene Technologies, Inc.

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