Anti-SPG7 Antibody (CAB10249)
- SKU:
- CAB10249
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-SPG7 Antibody |
| 抗体コード: | CAB10249 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1). |
| 申し込み: | WB IHC |
| 推奨希釈: | WB 1:1000 - 1:2000 IHC 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | U-87MG, MCF7, HT-29, K-562, Mouse brain, Mouse kidney, Mouse liver |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human SPG7 (NP_003110.1). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | MAVL LLLL RALR RGPG PGPR PLWG PGPA WSPG FPAR PGRG RPYM ASRP PGDL AEAG GRAL QSLQ LRLL TPTF EGIN GLLL KQHL VQNP VRLW QLLG GTFY FNTS RLKQ KNKE KDKS KGKA PEED EEER RRRE RDDQ MYRE RLRT LLVI AVVM SLLN ALST SGGS ISWN DFVH EMLA KGEV QRVQ VVPE SDVV EVYL HPGA VVFG RPRL ALMY RMQV ANID KFEE KLRA AEDE LNIE AKDR IPVS YKRT GF |
| 遺伝子ID: | 6687 |
| Uniprot: | Q9UQ90 |
| セルラーロケーション: | Mitochondrion membrane, Multi-pass membrane protein |
| 計算された分子量: | 53kDa/88kDa |
| 観察された分子量: | 88kDa |
| 同義語: | SPG7, CAR, CMAR, PGN, SPG5C |
| バックグラウンド: | This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. |
| UniProt Protein Function: | SPG7: Putative ATP-dependent zinc metalloprotease. Defects in SPG7 are the cause of spastic paraplegia autosomal recessive type 7 (SPG7). Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. SPG7 is a complex form. Additional clinical features are cerebellar syndrome, supranuclear palsy, and cognitive impairment, particularly disturbance of attention and executive functions. Defects in SPG7 may cause autosomal recessive osteogenesis imperfecta (OI). Osteogenesis imperfecta defines a group of connective tissue disorders characterized by bone fragility and low bone mass. Clinical features of SPG7-related osteogenesis imperfecta include recurrent fractures, mild bone deformities, delayed tooth eruption, normal hearing and white sclera. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Cell adhesion; Chaperone; EC 3.4.24.-; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Motility/polarity/chemotaxis; Protease Chromosomal Location of Human Ortholog: 16q24.3 Cellular Component: mitochondrion Molecular Function:metalloendopeptidase activity; peptidase activity; protein binding; unfolded protein binding Biological Process: nervous system development Disease: Spastic Paraplegia 7, Autosomal Recessive |
| NCBI Summary: | This gene encodes a mitochondrial metalloprotease protein that is a member of the AAA family. Members of this protein family share an ATPase domain and have roles in diverse cellular processes including membrane trafficking, intracellular motility, organelle biogenesis, protein folding, and proteolysis. Mutations in this gene cause autosomal recessive spastic paraplegia 7. Two transcript variants encoding distinct isoforms have been identified. [provided by RefSeq, Mar 2014] |
| UniProt Code: | Q9UQ90 |
| NCBI GenInfo Identifier: | 116242796 |
| NCBI Gene ID: | 6687 |
| NCBI Accession: | Q9UQ90.2 |
| UniProt Secondary Accession: | Q9UQ90,O75756, Q2TB70, Q58F00, Q96IB0, |
| UniProt Related Accession: | Q9UQ90 |
| Molecular Weight: | 88kDa |
| NCBI Full Name: | Paraplegin |
| NCBI Synonym Full Names: | SPG7, paraplegin matrix AAA peptidase subunit |
| NCBI Official Symbol: | SPG7 |
| NCBI Official Synonym Symbols: | CAR; PGN; CMAR; SPG5C |
| NCBI Protein Information: | paraplegin |
| UniProt Protein Name: | Paraplegin |
| UniProt Synonym Protein Names: | Spastic paraplegia 7 protein |
| Protein Family: | Paraplegin |
| UniProt Gene Name: | SPG7 |
 | Western blot analysis of extracts of various cell lines, using SPG7 antibody (CAB10249) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s. |
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