| 產(chǎn)品詳情 |
| Edit |   |
| Antigenic Specificity | Alpha-1-antitrypsin, Human |
| Clone | 2C1 |
| Host Species | Mouse |
| Reactive Species | human |
| Isotype | IgG1 |
| Format | unconjugated |
| Size | 0.5 mg |
| Concentration | n/a |
| Applications | immunoassay, IP, paraffin sections, WB |
| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
| Description | The mouse monoclonal antibody clone 2C1 recognizes polymeric forms of human alpha-1-antitrypsin. Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. Serpinopathies are conformational diseases characterized by the polymerization and intracellular retention of members of the serine protease inhibitor or serpin superfamily of proteins.1 The best known is a1-antitrypsin deficiency, with the most common severe deficiency allele being the Z mutation (Glu342Lys). The severe Z deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes. This causes neonatal hepatitis, cirrhosis, and hepatocellular carcinoma. . Clone 2C1 recognizes polymers formed by Z α1-antitrypsin in vivo. It also recognizes polymers formed by the Siiyama (Ser53Phe) and Brescia (Gly225Arg) mutants, and the novel His334Asp shutter domain mutant of a1-antitrypsin that is associated with prolonged neonatal jaundice in a 6-week-old boy. These data show that Z and shutter domain mutants form polymers with a shared epitope. |
| Immunogen | Z a1-antitrypsin polymers prepared from a1-antitrypsin purified from the plasma of PI*Z homozygotes |
| Other Names | Alpha-1 protease inhibitor, Alpha-1-antiproteinase, Serpin A1, A1AT. |
| Gene, Accession # | SERPINA1 |
| Catalog # | HM2289-IA |
| Price | |
| Order / More Info | Alpha-1-antitrypsin, Human Antibody from HYCULT BIOTECH |
| Product Specific References | n/a |
| 產(chǎn)品資料 |
|
日韩另类视频,手机看片一级片,欧美成人综合网站
|
成人无码www免费视频苹果版,男女深夜福利,五月婷婷色丁香
|
天天干夜夜探美女BB,淫欲护士日记,人人做人人操
|
操逼视频图,女技师三级做爰按摩电影,美国十次无码
|
青娱乐色色,欧洲极品丰满女同,亚洲九一
|
三级片小说,xxxxwwww日本泡妞,99精品一区二区三区的区别
|
影音先锋成人无码,啊轻点灬太粗嗯太深了用力太子妃,打床炮视频网站在线
|
AAAAAAA无码色情三级,操女大学生,人or猪or狗的dna视频
|
久草福利在线视频,youjizzxxxx18,影音先锋AV色情资源网
|
寡妇高潮一级毛片免费看,极品美女国产精品免费一区,操逼操逼操逼操逼操逼操逼
|
|