GLE1 Antibody, FITC conjugated (PACO36212)
- SKU:
- PACO36212
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | GLE1 Antibody, FITC conjugated (PACO36212) |
抗体コード: | PACO36212 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Nucleoporin GLE1 protein (141-380AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | 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). |
シノニム: | Nucleoporin GLE1 (hGLE1) (GLE1-like protein), GLE1, GLE1L |
UniProt Protein Function: | GLE1: 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). Defects in GLE1 are the cause of lethal congenital contracture syndrome type 1 (LCCS1); 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). 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. Belongs to the GLE1 family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Karyopherin; Nuclear export Chromosomal Location of Human Ortholog: 9q34.11 Cellular Component: extracellular space; membrane; cytoplasm; plasma membrane; nuclear pore Molecular Function:identical protein binding; protein binding Biological Process: poly(A)+ mRNA export from nucleus; mRNA export from nucleus; protein transport Disease: Lethal Arthrogryposis With Anterior Horn Cell Disease; Lethal Congenital Contracture Syndrome 1 |
UniProt Code: | Q53GS7 |
NCBI GenInfo Identifier: | 51317384 |
NCBI Gene ID: | 2733 |
NCBI Accession: | NP_001003722.1 |
UniProt Secondary Accession: | Q53GS7,O75458, Q53GT9, Q5VVU1, Q8NCP6, Q9UFL6, |
UniProt Related Accession: | Q53GS7 |
Molecular Weight: | 79,836 Da |
NCBI Full Name: | nucleoporin GLE1 isoform 1 |
NCBI Synonym Full Names: | GLE1 RNA export mediator |
NCBI Official Symbol: | GLE1 |
NCBI Official Synonym Symbols: | LCCS; GLE1L; LCCS1; hGLE1 |
NCBI Protein Information: | nucleoporin GLE1; GLE1-like protein; GLE1-like, RNA export mediator; GLE1 RNA export mediator homolog |
UniProt Protein Name: | Nucleoporin GLE1 |
UniProt Synonym Protein Names: | GLE1-like protein |
Protein Family: | Protein |
UniProt Gene Name: | GLE1 |
UniProt Entry Name: | GLE1_HUMAN |