Description
| 抗体名: | DNAJC19 Antibody (PACO45131) |
| 抗体コード: | PACO45131 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, WB, IHC, IP |
| 推奨される希釈: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200, IP:1:200-1:2000 |
| 反応性: | Human, Mouse |
| 免疫原: | Recombinant Human Mitochondrial import inner membrane translocase subunit TIM14 protein (1-116AA) |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 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 |
 | Western blot. All lanes: DNAJC19 antibody at 0.96µg/ml. Lane 1: Mouse kidney tissue. Lane 2: A549 whole cell lysate. Lane 3: HL60 whole cell lysate. Lane 4: Hela whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 13, 11 kDa. Observed band size: 13 kDa.. |
 | Immunohistochemistry of paraffin-embedded human kidney tissue using PACO45131 at dilution of 1:100. |
 | Immunohistochemistry of paraffin-embedded human brain tissue using PACO45131 at dilution of 1:100. |
| バックグラウンド: | Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity). |
| シノニム: | Mitochondrial import inner membrane translocase subunit TIM14 (DnaJ homolog subfamily C member 19), DNAJC19, TIM14 TIMM14 |
| UniProt Protein Function: | DNAJC19: Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity. Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5); also known as dilated cardiomyopathy with ataxia (DCMA). MGA5 is an autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure, and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid. Belongs to the TIM14 family. |
| UniProt Protein Details: | Protein type:Membrane protein, integral; Chaperone; Mitochondrial Chromosomal Location of Human Ortholog: 3q26.33 Cellular Component: protein complex; mitochondrion; mitochondrial inner membrane; integral to membrane Molecular Function:protein binding Biological Process: genitalia development; cellular protein metabolic process; protein folding; visual perception; protein targeting to mitochondrion Disease: 3-methylglutaconic Aciduria, Type V |
| NCBI Summary: | The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012] |
| UniProt Code: | Q96DA6 |
| NCBI GenInfo Identifier: | 74760780 |
| NCBI Gene ID: | 131118 |
| NCBI Accession: | Q96DA6.3 |
| UniProt Secondary Accession: | Q96DA6,B2R4B1, C9JBV1, |
| UniProt Related Accession: | Q96DA6 |
| Molecular Weight: | 116 |
| NCBI Full Name: | Mitochondrial import inner membrane translocase subunit TIM14 |
| NCBI Synonym Full Names: | DnaJ (Hsp40) homolog, subfamily C, member 19 |
| NCBI Official Symbol: | DNAJC19 |
| NCBI Official Synonym Symbols: | PAM18; TIM14; TIMM14 |
| NCBI Protein Information: | mitochondrial import inner membrane translocase subunit TIM14; homolog of yeast TIM14 |
| UniProt Protein Name: | Mitochondrial import inner membrane translocase subunit TIM14 |
| UniProt Synonym Protein Names: | DnaJ homolog subfamily C member 19 |
| UniProt Gene Name: | DNAJC19 |
| UniProt Entry Name: | TIM14_HUMAN |
