在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

Recombinant Human Delta-1-pyrroline-5-carboxylate synthase (ALDH18A1)

In Stock
  • 中文名稱:
    Recombinant Human Delta-1-pyrroline-5-carboxylate synthase(ALDH18A1)
  • 貨號(hào):
    CSB-EP001564HUe1
  • 規(guī)格:
    ¥2832
  • 圖片:
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他:

產(chǎn)品詳情

  • 純度:
    Greater than 85% as determined by SDS-PAGE.
  • 生物活性:
    Not Test
  • 基因名:
  • Uniprot No.:
  • 別名:
    (P5CS)(Aldehyde dehydrogenase family 18 member A1)(GK)(Gamma-glutamyl kinase)(GPR)(Glutamate-5-semialdehyde dehydrogenase)(Glutamyl-gamma-semialdehyde dehydrogenase)
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長(zhǎng)度:
    Full Length
  • 來源:
    E.coli
  • 分子量:
    87.3 kDa
  • 表達(dá)區(qū)域:
    1-795aa
  • 氨基酸序列
    MLSQVYRCGFQPFNQHLLPWVKCTTVFRSHCIQPSVIRHVRSWSNIPFITVPLSRTHGKSFAHRSELKHAKRIVVKLGSAVVTRGDECGLALGRLASIVEQVSVLQNQGREMMLVTSGAVAFGKQRLRHEILLSQSVRQALHSGQNQLKEMAIPVLEARACAAAGQSGLMALYEAMFTQYSICAAQILVTNLDFHDEQKRRNLNGTLHELLRMNIVPIVNTNDAVVPPAEPNSDLQGVNVISVKDNDSLAARLAVEMKTDLLIVLSDVEGLFDSPPGSDDAKLIDIFYPGDQQSVTFGTKSRVGMGGMEAKVKAALWALQGGTSVVIANGTHPKVSGHVITDIVEGKKVGTFFSEVKPAGPTVEQQGEMARSGGRMLATLEPEQRAEIIHHLADLLTDQRDEILLANKKDLEEAEGRLAAPLLKRLSLSTSKLNSLAIGLRQIAASSQDSVGRVLRRTRIAKNLELEQVTVPIGVLLVIFESRPDCLPQVAALAIASGNGLLLKGGKEAAHSNRILHLLTQEALSIHGVKEAVQLVNTREEVEDLCRLDKMIDLIIPRGSSQLVRDIQKAAKGIPVMGHSEGICHMYVDSEASVDKVTRLVRDSKCEYPAACNALETLLIHRDLLRTPLFDQIIDMLRVEQVKIHAGPKFASYLTFSPSEVKSLRTEYGDLELCIEVVDNVQDAIDHIHKYGSSHTDVIVTEDENTAEFFLQHVDSACVFWNASTRFSDGYRFGLGAEVGISTSRIHARGPVGLEGLLTTKWLLRGKDHVVSDFSEHGSLKYLHENLPIPQRNTN
    Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
    If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
  • 蛋白標(biāo)簽:
    Tag-Free
  • 產(chǎn)品提供形式:
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 緩沖液:
    If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
  • 復(fù)溶:
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲(chǔ)存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    3-7 business days
  • 注意事項(xiàng):
    Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.
  • Datasheet & COA:
    Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能:
    Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, ornithine and arginine.
  • 基因功能參考文獻(xiàn):
    1. Novel mutations in the ALDH18A1 gene in complicated hereditary spastic paraplegia with cerebellar ataxia and cognitive impairment. PMID: 29915212
    2. This is the first report of an individual with ALDH18A1-ADCL due to a substitution at a residue other than p.Arg138. Knowledge of the complete spectrum of dominant-acting mutations that cause this rare syndrome will have implications for molecular diagnosis and genetic counselling of these families. PMID: 28228640
    3. ALDH18A1 gene during vertebrate and invertebrate evolution and a proposal for generating the bifunctional vertebrate and invertebrate ALDH18A1 gene from a bacterial operon (proBA) encoding glutamyl kinase and glutamyl phosphate reductase. PMID: 27989597
    4. Recurrent De Novo Mutations Affecting Residue Arg138 of Pyrroline-5-Carboxylate Synthase Cause a Progeroid Form of Autosomal-Dominant Cutis Laxa. PMID: 26320891
    5. autosomal recessive transmission of ALDH18A1 mutations, and predominant complex hereditary spastic paraplegia with marked cognitive impairment PMID: 26026163
    6. A frameshift deletion of one nucleotide and a microdeletion affecting the ALDH18A1 gene, respectively, in a homozygous state in both patients, was identified. PMID: 24913064
    7. expansion of the phenotypic spectrum associated with mutations in ALDH18A1 PMID: 21739576
    8. ALDH18A1 genetic variants are associated with Down syndrome in subjects with dementia of Alzheimer's disease. PMID: 20946940
    9. analysis of function and regulation of Delta1-pyrroline-5-carboxylate synthase PMID: 18401542
    10. These data suggest that P5CS may possess additional uncharacterised functions that affect connective tissue and central nervous system function. PMID: 18478038

    顯示更多

    收起更多

  • 相關(guān)疾病:
    Cutis laxa, autosomal recessive, 3A (ARCL3A); Cutis laxa, autosomal dominant, 3 (ADCL3); Spastic paraplegia 9A, autosomal dominant (SPG9A); Spastic paraplegia 9B, autosomal recessive (SPG9B)
  • 亞細(xì)胞定位:
    Mitochondrion inner membrane.
  • 蛋白家族:
    Glutamate 5-kinase family; Gamma-glutamyl phosphate reductase family
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 9722

    OMIM: 138250

    KEGG: hsa:5832

    STRING: 9606.ENSP00000360268

    UniGene: Hs.500645



主站蜘蛛池模板: 久久久久成人精品| 邪恶肉肉全彩色无遮盖| 久久亚洲国产精品五月天婷| 国产农村熟妇videos| 国产精品青青在线观看爽香蕉| 狼友av永久网站免费观看孕交| 国产精品自在拍首页视频 | 精品无码国产一区二区三区麻豆| 中文幕无线码中文字蜜桃| 国产精品无码2021在线观看 | 极品少妇被黑人白浆直流| 亚洲综合网站久久久| 天天做天天爱夜夜爽毛片毛片| 亚洲日韩精品欧美一区二区一| 无码欧美黑人xxx一区二区三区 | 亚洲 欧洲 日韩 综合av| 国产成人一区二区三区在线| 男女啪啪做爰高潮无遮挡| 大伊香蕉在线精品视频75| 宝宝好涨水快流出来免费视频| 久久久精品波多野结衣| 丝袜无码专区人妻视频| 精品国产一区二区三区av色诱| 色视频www在线播放国产人成| 国产精品人妻| 免费看成人午夜福利专区| 日本边添边摸边做边爱边| 成年女人毛片免费视频| 国产性猛交╳xxx乱大交| 99久久国产亚洲高清观看| 欧洲精品免费一区二区三区| 富婆如狼似虎找黑人老外| 日韩精品无码久久久久久| 亚洲一区二区av在线观看| 色偷偷888欧美精品久久久| 国产高清午夜人成在线观看| 午夜性爽视频男人的天堂| 日本熟妇人妻xxxx| 国产精品成人永久在线四虎| 亚洲女教师丝祙在线播放| 亚洲精品久久久久午夜aⅴ|