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Invitrogen
Complement Factor Bb Monoclonal Antibody (10-09)
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产品信息
MA5-28085
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG1
分类
Monoclonal
类型
Antibody
克隆号
10-09
抗原
Human factor B
偶联物
形式
Liquid
浓度
1.05 mg/mL
纯化类型
Protein A
保存液
BBS
内含物
0.1% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Ambient (domestic); Wet ice (international)
RRID
产品详细信息
This antibody recognizes the Bb fragment of 90 kDa human complement factor B, present in blood serum. C3b associates with complement factor B, inducing conformational change which enables complement factor D to cleave the N-terminal of complement factor B, the Ba subunit, leaving the 63 kDa Bb subunit associated with C3b and forming C3 convertase. Subunit Bb is involved in the proliferation of preactivated B lymphocytes. Conversely, Ba inhibits lymphocyte proliferation.
靶标信息
CFB is involved in hemolytic uremic syndrome atypical 4 (AHUS4), an atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Disease susceptibility is associated with variations affecting the gene represented in this entry. Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: B-factor, properdin; C3 proaccelerator; C3 proactivator; C3/C5 convertase; Complement factor B; GBG; Glycine-rich beta glycoprotein; glycine-rich beta-glycoprotein; PBF2; Properdin factor B
基因别名: AHUS4; ARMD14; BF; BFD; CFAB; CFB; CFBD; FB; FBI12; GBG; H2-Bf; PBF2
UniProt ID: (Human) Q9BX92
Entrez Gene ID: (Human) 629
