Anti-AFG3L2 Antibody (CAB15393)
- SKU:
- CAB15393
- 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-AFG3L2 Antibody |
| 抗体コード: | CAB15393 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 538-797 of human AFG3L2 (NP_006787.2). |
| 申し込み: | WB IHC IF |
| 推奨希釈: | WB 1:200 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | LO2, 293T, HeLa, Mouse brain, Mouse heart, Mouse kidney, Mouse liver, Mouse pancreas |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 538-797 of human AFG3L2 (NP_006787.2). |
| 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: | DSIN QKHF EQAI ERVI GGLE KKTQ VLQP EEKK TVAY HEAG HAVA GWYL EHAD PLLK VSII PRGK GLGY AQYL PKEQ YLYT KEQL LDRM CMTL GGRV SEEI FFGR ITTG AQDD LRKV TQSA YAQI VQFG MNEK VGQI SFDL PRQG DMVL EKPY SEAT ARLI DDEV RILI NDAY KRTV ALLT EKKA DVEK VALL LLEK EVLD KNDM VELL GPRP FAEK STYE EFVE GTGS LDED TSLP EGLK DWNK EREK EKEE PPGE KVAN |
| 遺伝子ID: | 10939 |
| Uniprot: | Q9Y4W6 |
| セルラーロケーション: | Mitochondrion membrane, Multi-pass membrane protein |
| 計算された分子量: | 88kDa |
| 観察された分子量: | 89kDa |
| 同義語: | AFG3L2, SCA28, SPAX5 |
| バックグラウンド: | This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders. |
| UniProt Protein Function: | AFG3L2: ATP-dependent protease which is essential for axonal development. Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28). It is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA28 is an autosomal dominant cerebellar ataxia (ADCA) with a slow progressive course and no evidence of sensory involvement or cognitive impairment. Defects in AFG3L2 are the cause of spastic ataxia autosomal recessive type 5 (SPAX5). A neurodegenerative disorder characterized by early onset spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. |
| UniProt Protein Details: | Protein type:Protease; EC 3.4.24.-; Chaperone; Membrane protein, multi-pass; Mitochondrial; Membrane protein, integral; Cell development/differentiation Chromosomal Location of Human Ortholog: 18p11 Cellular Component: mitochondrial inner membrane; mitochondrion Molecular Function:ATP-dependent peptidase activity; metallopeptidase activity; protein binding; unfolded protein binding Biological Process: cristae formation; mitochondrial fusion; nerve development; protein complex assembly; protein import into mitochondrial intermembrane space Disease: Spastic Ataxia 5, Autosomal Recessive; Spinocerebellar Ataxia 28 |
| NCBI Summary: | This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders. [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q9Y4W6 |
| NCBI GenInfo Identifier: | 126302516 |
| NCBI Gene ID: | 10939 |
| NCBI Accession: | Q9Y4W6.2 |
| UniProt Secondary Accession: | Q9Y4W6,Q6P1L0, |
| UniProt Related Accession: | Q9Y4W6 |
| Molecular Weight: | 88,584 Da |
| NCBI Full Name: | AFG3-like protein 2 |
| NCBI Synonym Full Names: | AFG3 like matrix AAA peptidase subunit 2 |
| NCBI Official Symbol: | AFG3L2 |
| NCBI Official Synonym Symbols: | SCA28; SPAX5 |
| NCBI Protein Information: | AFG3-like protein 2 |
| UniProt Protein Name: | AFG3-like protein 2 |
| UniProt Synonym Protein Names: | Paraplegin-like protein |
| Protein Family: | AFG3-like protein |
| UniProt Gene Name: | AFG3L2 |
| UniProt Entry Name: | AFG32_HUMAN |
 | Western blot analysis of extracts of various cell lines, using AFG3L2 antibody (CAB15393) 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: 5s. |
 | Immunohistochemistry of paraffin-embedded rat brain using AFG3L2 antibody (CAB15393) at dilution of 1:200 (40x lens). |
 | Immunohistochemistry of paraffin-embedded human appendix using AFG3L2 antibody (CAB15393) at dilution of 1:200 (40x lens). |
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