超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
美女扒开内裤无遮挡18禁,在线一区二区三区精品,国产又粗又黄又猛又爽的视频
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-Galactosylceramidase/AP Conjugated antibody (bs-13251R-AP)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13251R-AP
英文名稱 Rabbit Anti-Galactosylceramidase/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的半乳糖神經酰胺酶抗體
別    名 Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 73kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human G protein-regulated inducer of neurite outgrowth 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 癈 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癈. 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 癈.
產品介紹 background:
GALC is a lysosomal enzyme that hydrolyzes galactose ester bonds in various galactolipids, including galactosylceramide, galactosylsphingosine, lactosylceramide and monogalactosyldiglyceride. Galactolipids contain glucose and/or galactose, and are found in the brain and other nerve tissue, especially the myelin sheath. Galactosylceramide is a major lipid in myelin, kidney, and epithelial cells of the small intestine and colon. Mutations in the GALC gene that compromise protein function correlate to Krabbe disease (globoid cell leukodystrophy, GLD). GLD is an autosomal recessive condition that affects approximately 1 in 150,000 infants and results in progressive destruction of the nervous system. The “twitcher” mouse is a model system for GLD; the genotype is a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.

Function:
Galactosylceramidase hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is an enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. It shows highest level of activity in testes compared to brain, kidney, placenta and liver. It can also be found in urine. Defects in Galactosylceramidase are the cause of globoid cell leukodystrophy (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified.

Subcellular Location:
Lysosomal.

Tissue Specificity:
Highest level of activity in testes compared to brain, kidney, placenta and liver. Can also be found in urine.

DISEASE:
Defects in GALC are the cause of leukodystrophy globoid cell (GLD) [MIM:245200]; also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified.

Similarity:
Belongs to the glycosyl hydrolase 59 family.

Database links:

Entrez Gene: 533428 Cow

Entrez Gene: 403916 Dog

Entrez Gene: 2581 Human

Entrez Gene: 14420 Mouse

Entrez Gene: 314360 Rat

Omim: 606890 Human

SwissProt: P54804 Dog

SwissProt: P54803 Human

SwissProt: P54818 Mouse

Unigene: 41497 Cow

Unigene: 3777 Dog

Unigene: 513439 Human

Unigene: 5120 Mouse

Unigene: 30608 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.ggggww.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 免费观看黄色网页 | 国产精品一区久久看 | 国产女人被狂躁到高潮小说 | 乱码精品国产成人观看免费 | 欧美日韩国产第一页 | 午夜精品久久久久久久99 | 日韩视频精品在线 | 欧美中文字幕一区二区三区亚洲 | 熟妇的奶头又大又长奶水视频 | 精品久久久久久中文字幕2017 | 欧美美乳视频网站在线观看 | 成人在线免费看 | 18禁美女裸体网站无遮挡 | 国产亚洲影视在线视频播放 | 四虎海外网址 | 美女h片 | 手机午夜福利1000视频 | 国产成人无码精品久久灭火器 | 亚洲成人三级在线观看 | 国产九区一区在线 | 丝袜老师张开腿任我玩弄 | 国产网战无遮挡 | 国模两腿玉门打开图无码 | 国产gv在线观看受被做哭 | 插插天天 | 亚洲综合无码精品一区二区三区 | 永久免费av在线观看 | 欧美最猛黑人xxxx黑人猛叫黄 | 天天摸天天操视频 | 国产一级一级理论片一区二区 | 久久综合成人网 | 亚洲精品禁 | 国产免费无遮挡吸乳视频 | 国产精品久久久国产 | 中文字幕国产亚洲 | 日韩精品一区二区三区丰满 | 懂色中文一区二区在线播放 | 久久久久亚洲波多野结衣 | 999精品欧美一区二区三区黑人 | 久久精品国产91精品亚洲高清 | 浓毛熟女看18p大黑p |