PANK2 Antibody (PACO48746)
- SKU:
- PACO48746
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | PANK2 Antibody (PACO48746) |
抗体コード: | PACO48746 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC, IF |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:20-1:200, IF:1:50-1:200 |
反応性: | Human, Mouse |
免疫原: | Recombinant Human Pantothenate kinase 2, mitochondrial protein (437-490AA) |
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. Positive WB detected in: Mouse liver tissue, Mouse kidney tissue. All lanes: PANK2 antibody at 3.4µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 63, 31, 50, 51 kDa. Observed band size: 63, 31 kDa.. | |
Immunofluorescent analysis of HepG2 cells using PACO48746 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). | |
Immunohistochemistry of paraffin-embedded human colon cancer using PACO48746 at dilution of 1:100. |
バックグラウンド: | May be the master regulator of the CoA biosynthesis. |
シノニム: | Pantothenate kinase 2, mitochondrial (hPanK2) (EC 2.7.1.33) (Pantothenic acid, kinase 2), PANK2, C20orf48 |
UniProt Protein Function: | PANK2: a pantothenate kinase that catalyzes the first committed step in the biosynthesis of coenzyme A, an essential cofactor in cellular metabolism. PANK2 is predominantly localized in mitochondria in primates. Defects in PANK2 are the cause of pantothenate kinase-associated neurodegeneration (PKAN), an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain. Three isoforms of the human PANK2, produced by alternative splicing, have been reported. |
UniProt Protein Details: | Protein type:Mitochondrial; Kinase, other; EC 2.7.1.33; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis Chromosomal Location of Human Ortholog: 20p13 Cellular Component: cytoplasm; mitochondrial intermembrane space Molecular Function:pantothenate kinase activity Biological Process: coenzyme biosynthetic process; regulation of fatty acid metabolic process Disease: Hypoprebetalipoproteinemia, Acanthocytosis, Retinitis Pigmentosa, And Pallidal Degeneration; Neurodegeneration With Brain Iron Accumulation 1 |
NCBI Summary: | This gene encodes a protein belonging to the pantothenate kinase family and is the only member of that family to be expressed in mitochondria. Pantothenate kinase is a key regulatory enzyme in the biosynthesis of coenzyme A (CoA) in bacteria and mammalian cells. It catalyzes the first committed step in the universal biosynthetic pathway leading to CoA and is itself subject to regulation through feedback inhibition by acyl CoA species. Mutations in this gene are associated with HARP syndrome and pantothenate kinase-associated neurodegeneration (PKAN), formerly Hallervorden-Spatz syndrome. Alternative splicing, involving the use of alternate first exons, results in multiple transcripts encoding different isoforms. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q9BZ23 |
NCBI GenInfo Identifier: | 118572682 |
NCBI Gene ID: | 80025 |
NCBI Accession: | Q9BZ23.3 |
UniProt Secondary Accession: | Q9BZ23,Q5T7I2, Q5T7I4, Q7RTX5, Q8N7Q4, Q8TCR5, Q9BYW5 Q9HAF2, B1AK33, B2Z3X0, D3DVZ0, |
UniProt Related Accession: | Q9BZ23 |
Molecular Weight: | 50,582 Da |
NCBI Full Name: | Pantothenate kinase 2, mitochondrial |
NCBI Synonym Full Names: | pantothenate kinase 2 |
NCBI Official Symbol: | PANK2 |
NCBI Official Synonym Symbols: | HSS; HARP; PKAN; NBIA1; C20orf48 |
NCBI Protein Information: | pantothenate kinase 2, mitochondrial |
UniProt Protein Name: | Pantothenate kinase 2, mitochondrial |
UniProt Synonym Protein Names: | Pantothenic acid kinase 2 |
Protein Family: | Pantothenate kinase |
UniProt Gene Name: | PANK2 |
UniProt Entry Name: | PANK2_HUMAN |