AFG3L2 Antibody (PACO31796)
- SKU:
- PACO31796
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | AFG3L2 Antibody (PACO31796) |
抗体コード: | PACO31796 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200 |
反応性: | Human |
免疫原: | Recombinant Human AFG3-like protein 2 protein (550-759AA) |
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: | Non-conjugated |
![]() | Western blot All lanes: AFG3L2 antibody at 2µg/ml + EC109 whole cell lysate Secondary Goat polyclonal to rabbit IgG at 1/15000 dilution Predicted band size: 89 kDa Observed band size: 89 kDa . |
![]() | Immunohistochemistry of paraffin-embedded human prostate tissue using PACO31796 at dilution of 1:100. |
バックグラウンド: | ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU. Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form (By similarity). |
シノニム: | AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein), AFG3L2 |
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 |