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GLE1 Rabbit pAb

GLE1 Rabbit pAb

     
  • 14 - GLE1 Rabbit pAb AP55147
    Paraformaldehyde-fixed, paraffin embedded (rat testis); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (GLE1) Polyclonal Antibody, Unconjugated (AP55147) at 1:5000 overnight at 4°C, followed by a conjugated secondary (sp-0023) for 20 minutes and DAB staining.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF
Primary Accession Q53GS7
Reactivity Rat
Predicted Pig, Dog, Human, Mouse, Horse, Sheep
Host Rabbit
Clonality Polyclonal
Calculated MW 79836 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human GLE1
Epitope Specificity 611-698/698
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Nucleus. Cytoplasm. Shuttles between the nucleus and the cytoplasm. Shuttling is essential for its mRNA export function and Cytoplasm. Nucleus > nuclear pore complex. Shuttles between the nucleus and the cytoplasm. In the nucleus, isoform 1 localizes to the nuclear pore complex and nuclear envelope. Shuttling is essential for its mRNA export function.
DISEASE Defects in GLE1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1) [MIM:253310]; also known as multiple contracture syndrome type Finnish. LCCS is an autosomal recessive disorder characterized by early fetal hydrops and akinesia, micrognatia, pulmonary hypoplasia, pterygia, multiple joint contractures, specific neuropathology with degeneration of anterior horn neurons and extreme skeletal muscle atrophy. LCCS1 leads to prenatal death. Defects in GLE1 are the cause of lethal arthrogryposis with anterior horn cell disease (LAAHD) [MIM:611890]. LAAHD is characterized by fetal akinesia, arthrogryposis and motor neuron loss. LAADH fetus often survive delivery, but die early as a result of respiratory failure. Neuropathological findings resemble those of LCCS1, but are less severe.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions Protein transport across the nucleus is a selective, multi-step process involving several cytoplasmic factors that mediate protein passage through the nuclear pore complex (NPC). Gle1, also known as GLE1L, is a 698 amino acid protein that localizes to both the nucleus and the cytoplasm and belongs to the Gle1 family. Expressed as two alternatively spliced isoforms, Gle1 associates with the NPC and is required for the transport of poly(A)-containing mRNAs from the nucleus to the cytoplasm. Defects in the gene encoding Gle1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1) and lethal arthrogryposis with anterior horn cell disease (LAAHD), the former of which is characterized by early fetal hydrops and akinesia, micrognatia, pulmonary hypoplasia, pterygia and prenatal death, while the latter is associated with respiratory failure.
Additional Information
Gene ID 2733
Other Names mRNA export factor GLE1, hGLE1, GLE1 RNA export mediator {ECO:0000303|PubMed:18204449, ECO:0000312|HGNC:HGNC:4315}, GLE1-like protein, Nucleoporin GLE1, GLE1, GLE1L
Dilution IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name GLE1
Synonyms GLE1L
Function Required for the export of mRNAs containing poly(A) tails from the nucleus into the cytoplasm. May be involved in the terminal step of the mRNA transport through the nuclear pore complex (NPC).
Cellular Location Nucleus. Cytoplasm. Note=Shuttles between the nucleus and the cytoplasm (PubMed:12668658). Shuttling is essential for its mRNA export function (PubMed:12668658).
Research Areas

BACKGROUND

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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