Anti-HPS4 Antibody (CAB16157)
- SKU:
- CAB16157
- 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-HPS4 Antibody |
| 抗体コード: | CAB16157 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | A synthetic peptide corresponding to a sequence within amino acids 550-650 of human HPS4 (NP_690054.1). |
| 申し込み: | WB IF |
| 推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | U-87MG, SGC-7901, LO2, Jurkat, Mouse liver |
| 免疫原: | A synthetic peptide corresponding to a sequence within amino acids 550-650 of human HPS4 (NP_690054.1). |
| 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: | LLAE EPLL GDSA AIEE VYHS SLAS LNGL EVHL KETL PRDE AAST SSTY NFTH YDRI QSLL MANL PQVA TPQD RRFL QAVS LMHS EFAQ LPAL YEMT VRNA S |
| 遺伝子ID: | 89781 |
| Uniprot: | Q9NQG7 |
| セルラーロケーション: | |
| 計算された分子量: | 58kDa/76kDa/78kDa |
| 観察された分子量: | 115kDa |
| 同義語: | HPS4, BLOC3S2, LE |
| バックグラウンド: | This gene encodes a protein component of biogenesis of lysosome-related organelles complexes (BLOC). BLOC complexes are important for the formation of endosomal-lysosomal organelles such as melanosomes and platelet dense granules. Mutations in this gene result in subtype 4 of Hermansky-Pudlak syndrome, a form of albinism. Alternative splicing results in multiple transcript variants. |
| UniProt Protein Function: | HPS4: May function in the pathway of organelle biogenesis. Defects in HPS4 are the cause of Hermansky-Pudlak syndrome type 4 (HPS4). Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. 4 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Chromosomal Location of Human Ortholog: 22cen-q12.3 Cellular Component: platelet dense granule; membrane; lysosome; cytoplasm; melanosome Molecular Function:protein dimerization activity; protein binding; protein homodimerization activity Biological Process: positive regulation of eye pigmentation; protein stabilization; lysosome organization and biogenesis; hemostasis; melanocyte differentiation; blood coagulation; protein targeting Disease: Hermansky-pudlak Syndrome 4 |
| NCBI Summary: | This gene encodes a protein component of biogenesis of lysosome-related organelles complexes (BLOC). BLOC complexes are important for the formation of endosomal-lysosomal organelles such as melanosomes and platelet dense granules. Mutations in this gene result in subtype 4 of Hermansky-Pudlak syndrome, a form of albinism. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2012] |
| UniProt Code: | Q9NQG7 |
| NCBI GenInfo Identifier: | 21362617 |
| NCBI Gene ID: | 89781 |
| NCBI Accession: | Q9NQG7.2 |
| UniProt Secondary Accession: | Q9NQG7,Q5H8V6, Q96LX6, Q9BY93, Q9UH37, Q9UH38, B1AHQ4 |
| UniProt Related Accession: | Q9NQG7 |
| Molecular Weight: | 78,648 Da |
| NCBI Full Name: | Hermansky-Pudlak syndrome 4 protein |
| NCBI Synonym Full Names: | Hermansky-Pudlak syndrome 4 |
| NCBI Official Symbol: | HPS4 |
| NCBI Official Synonym Symbols: | LE |
| NCBI Protein Information: | Hermansky-Pudlak syndrome 4 protein; light-ear protein homolog |
| UniProt Protein Name: | Hermansky-Pudlak syndrome 4 protein |
| UniProt Synonym Protein Names: | Light-ear protein homolog |
| Protein Family: | Hermansky-Pudlak syndrome 4 protein |
| UniProt Gene Name: | HPS4 |
| UniProt Entry Name: | HPS4_HUMAN |
 | Western blot analysis of extracts of various cell lines, using HPS4 antibody (CAB16157) 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: 1s. |
 | Immunofluorescence analysis of C6 cells using HPS4 antibody (CAB16157) at dilution of 1:100. Blue: DAPI for nuclear staining. |
 | Immunofluorescence analysis of L929 cells using HPS4 antibody (CAB16157) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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