ATL1 Antibody (PACO07909)
- SKU:
- PACO07909
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | ATL1 Antibody (PACO07909) |
抗体コード: | PACO07909 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human ATL1 |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | atlastin GTPase 1;ATL1;AD-FSP;FSP1;GBP3;SPG3;SPG3A;atlastin1 ; |
UniProt Protein Function: | atlastin: GTPase tethering membranes through formation of trans- homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development. Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3); also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Defects in ATL1 are the cause of hereditary sensory neuropathy type 1D (HSN1D). HSN1D is a disease characterized by adult-onset distal axonal sensory neuropathy leading to mutilating ulcerations as well as hyporeflexia. Some patients may show features suggesting upper neuron involvement. Belongs to the GBP family. Atlastin subfamily. |
UniProt Protein Details: | Protein type:Membrane protein, integral; Membrane protein, multi-pass; Vesicle; EC 3.6.5.- Chromosomal Location of Human Ortholog: 14q22.1 Cellular Component: Golgi membrane; Golgi apparatus; endoplasmic reticulum membrane; axon; endoplasmic reticulum; integral to membrane; Golgi cis cisterna Molecular Function:GTPase activity; identical protein binding; protein binding; GTP binding Biological Process: endoplasmic reticulum organization and biogenesis; axonogenesis; metabolic process; protein homooligomerization Disease: Neuropathy, Hereditary Sensory, Type Id; Spastic Paraplegia 3, Autosomal Dominant |
NCBI Summary: | The protein encoded by this gene is a GTPase and a Golgi body transmembrane protein. The encoded protein can form a homotetramer and has been shown to interact with spastin and with mitogen-activated protein kinase kinase kinase kinase 4. This protein may be involved in axonal maintenance as evidenced by the fact that defects in this gene are a cause of spastic paraplegia type 3. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q8WXF7 |
NCBI GenInfo Identifier: | 37999727 |
NCBI Gene ID: | 51062 |
NCBI Accession: | Q8WXF7.1 |
UniProt Secondary Accession: | Q8WXF7,O95890, Q69YH7, Q96FK0, A6NND5, A8K2C0, G5E9T1 |
UniProt Related Accession: | Q8WXF7 |
Molecular Weight: | 63,055 Da |
NCBI Full Name: | Atlastin-1 |
NCBI Synonym Full Names: | atlastin GTPase 1 |
NCBI Official Symbol: | ATL1 |
NCBI Official Synonym Symbols: | FSP1; GBP3; SPG3; HSN1D; SPG3A; AD-FSP; atlastin1 |
NCBI Protein Information: | atlastin-1; GBP-3; hGBP3; GTP-binding protein 3; guanylate-binding protein 3; spastic paraplegia 3 protein A; brain-specific GTP-binding protein; guanine nucleotide-binding protein 3 |
UniProt Protein Name: | Atlastin-1 |
UniProt Synonym Protein Names: | Brain-specific GTP-binding protein; GTP-binding protein 3; GBP-3; hGBP3; Guanine nucleotide-binding protein 3; Spastic paraplegia 3 protein A |
Protein Family: | Atlastin |
UniProt Gene Name: | ATL1 |
UniProt Entry Name: | ATLA1_HUMAN |