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1,6-二磷酸果糖鎂鹽   
英文名稱：FDPMg；D-Fructose-1,6-diphoshate maesium salt   
其他名稱：1,6-二磷酸左旋糖鎂鹽；D-果糖-1,6-二磷酸鎂鹽；果糖-1,6-二磷酸鎂鹽；D-果糖-1,6-二磷酸鎂鹽   
C6H11Mg1.5O12P2 ·6H2O=481.45   
級別：BR   
FDPH4含量：≥70%   
Mg含量：7～10%   
重金屬：≤10ppm   
性狀(以下信息僅供參考)：白色或黃色粉末   
用途：本品僅供科研，不得用于其它用途   
保存：2～8℃1,6-二磷酸果糖鎂鹽儲存條件：
避光、干燥陰涼處封閉貯存,嚴(yán)禁與有毒、有害物品混放、混運。本品為非危險 產(chǎn)品可按一般化學(xué)品運輸,輕搬動輕放,防止日曬、雨淋！受熱、受潮、受光后易喪失活力,保存期短,因此貯存和運輸條件比較苛刻。
運輸：汽車運輸、EMS郵政快遞，申通快遞等， 款到上海3天內(nèi)發(fā)貨；
售后：如您對我們的產(chǎn)品服務(wù)及技術(shù)指標(biāo)有特殊要求，請及時通知我方。
存儲：應(yīng)貯存在干燥清潔避光的環(huán)境中，嚴(yán)禁與有毒物質(zhì)混放，以免污染（保質(zhì)期為兩年）。
1,6-二磷酸果糖鎂鹽主要優(yōu)級純、分級純和化學(xué)純3種：
（1）優(yōu)級純（GR：Guaranteed reagent），又稱一級品或保證試劑，99.8%，這種試劑純度Z高，雜質(zhì)含量Z低，適合于重要精密的分析工作和科學(xué)研究工作，使用綠色瓶簽。
（2）分析純（AR），又稱二級試劑，純度很高，99.7%，略次于優(yōu)級純，適合于重要分析及一般研究工作，使用紅色瓶簽。
（3）化學(xué)純（CP），又稱三級試劑，≥ 99.5%，純度與分析純相差較大，適用于工礦、學(xué)校一般分析工作。使用藍色（深藍色）標(biāo)簽。
（4）實驗試劑（LR：Laboratory reagent），又稱四級試劑。
1Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 Atlastins are Golgi-localized, integral membrane proteins that function as GTPases. The Atlastin proteins, also designated SPG3A and guanylate-binding protein 3, comprise a Dynamin superfamily that plays a role in axonal maintenance. Hereditary spastic paraplegia (HSP) is an inherited neurodegenerative disorder that is characterized by retrograde axonal degeneration. HSP primarily affects long corticospinal neurons and causes spastic lower extremity weakness. Spastin, a microtubule (MT)-severing AAA ATPase, is a binding partner of Atlastin that is involved in membrane dynamics. This Spastin/Atlastin binding may be involved in the biochemical pathway that leads to HSP development. Mutations in the Atlastin gene (SPG3A) account for approximay 10% of all autosomal dominant HSPs, while mutations in the Spastin gene (SPG4) account for almost 40%.
Function : GTPase tethering membranes through formation of trans-homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development.
Subunit : Homooligomer. Interacts (via N-terminal region) withMAP4K4 (via CNH regulatory domain). Interacts with REEP5, RTN3 andRTN4 (via the transmembrane region). Interacts with SPAST;interaction is direct. May interact with TMED2. Interacts withREEP1.
Subcellular Location : Endoplasmic reticulum membrane. Golgi apparatus membrane. Cell projection > axon.
Tissue Specificity : Expressed predominantly in the adult and fetal central nervous system. Measurable expression in all tissues examined, although expression in adult brain is at least 50-fold higher than in other tissues. Detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain. Expressed in upper and lower motor neurons (at protein level).
DISEASE : Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3) [MIM:182600]; also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.
Similarity : Belongs to the GBP family. Atlastin subfamily.
Database links : UniProtKB/Swiss-Prot: Q8F7.1
英文名稱  Anti-SPG48
中文名稱  SPG48蛋白抗體
別    名  Hypothetical protein LOC9907; KIAA0415; Uncharacterized protein KIAA0415; AP5Z1_HUMAN.
濃    度  1mg/1ml
規(guī) 格  0.2ml/200μg
抗體來源  Rabbit 
克隆類型  polyclonal
交叉反應(yīng)  Human
產(chǎn)品類型  一抗   
研究領(lǐng)域  細(xì)胞生物 神經(jīng)生物學(xué) 表觀遺傳學(xué) 
蛋白分子量  predicted molecular weight: 89kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated synthetic peptide derived from human SPG48 (288-370aa)
亞    型  IgG
純化方法  affinity purified by Protein A
儲 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
產(chǎn)品應(yīng)用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500
（石蠟切片需做抗原修復(fù)）
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user. 
保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 Chromosome 7 is about 158 milllion bases long, encodes over 1000 genes and makes up about 5% of the human genome. Chromosome 7 has been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. The deletion of a portion of the q arm of chromosome 7 is associated with Williams-Beuren syndrome, a condition characterized by mild mental retardation, an unusual comfort and friendliness with strangers and an elfin appearance. Deletions of portions of the q arm of chromosome 7 are also seen in a number of myeloid disorders including cases of acute myelogenous leukemia and myelodysplasia. KIAA0415 is a 807 amino acid protein that exists as three alternatively spliced isoforms. The KIAA0415 gene product has been provisionally designated KIAA0415 pending further characterization.
Function : The function of SPG48 remains unknown. There are 3 named isoforms produced by alternative splicing.
Subunit : Probably part of the adapter protein complex 5 (AP-5) atetramer composed of AP5B1, AP5M1, AP5S1 and AP5Z1. Interacts withZFYVE26 and SPG11.
Subcellular Location : Cytoplasm. Nucleus. Note=By SDS-PAGE, 2isoforms have been observed, the shorter seems to be predominantlynuclear and the longer is mostly cytoplasmic (PubMed:20613862).
DISEASE : Defects in AP5Z1 are the cause of spastic paraplegiaautosomal recessive type 48 (SPG48) [MIM:613647]. A form of spasticparaplegia, a neurodegenerative disorder characterized by a slow,gradual, progressive weakness and spasticity of the lower limbs.Rate of progression and the severity of symptoms are quitevariable. Initial symptoms may include difficulty with balance,weakness and stiffness in the legs, muscle spasms, and dragging thetoes when walking. In some forms of the disorder, bladder symptoms(such as incontinence) may appear, or the weakness and stiffnessmay spread to other parts of the body.
Database links : UniProtKB/Swiss-Prot: O43299.2
英文名稱  Anti-SPG7/Paraplegin
中文名稱  痙攣性截癱蛋白7/基質(zhì)細(xì)胞粘附調(diào)節(jié)蛋白抗體
別    名  CAR; Cell adhesion regulator; Cell matrix adhesion regulator; CMAR; Paraplegin; PGN; Spastic paraplegia 7 (pure and complicated autosomal recessive); Spastic paraplegia 7 homolog (human); Spastic paraplegia 7, paraplegin (pure and complicated autosomal recessive); Spastic paraplegia protein 7; SPG5C; SPG7 gene; SPG7_HUMAN.
濃    度  1mg/1ml
規(guī) 格  0.2ml/200μg
抗體來源  Rabbit 
克隆類型  polyclonal
交叉反應(yīng)  Human, Mouse, Rat
產(chǎn)品類型  一抗   
研究領(lǐng)域  心血管 細(xì)胞生物 神經(jīng)生物學(xué) 細(xì)胞粘附分子 
蛋白分子量  predicted molecular weight: 88kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated synthetic peptide derived from human Paraplegin (201-285aa)
亞    型  IgG
純化方法  affinity purified by Protein A
儲 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
產(chǎn)品應(yīng)用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500