C1Q,补体C1qα链多肽抗体

产品编号HZ-10795R
英文名称C1Q
中文名称补体C1qα链多肽抗体
别 名C1QA; C1QA_HUMAN; C1QB; C1QC; Complement C1q subcomponent subunit A; Complement C1q subcomponent subunit B; Complement C1q subcomponent subunit C; Complement component 1 q subcomponent A chain; Complement component 1 q subcomponent alpha polypeptide; Complement component C1q A chain; Complement component C1q B chain; Complement component C1q C chain.
说 明 书0.1ml 0.2ml
研究领域
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Mouse, Rat,
C1Q,补体C1qα链多肽抗体产品应用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 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量24kDa
细胞定位分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from mouse C1Q
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件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.
C1Q,补体C1qα链多肽抗体PubMedPubMed
产品介绍background:
C1q, a subcomponent of the classical complement pathway, is composed of nine subunits that mediate classical complement activation and thereby play an important role in the immune response. Six of these subunits are disulfide-linked dimers of chains A and B, while three of these subunits, designated C1q-A through C1q-C, are disulfide-linked dimers of chain C. The presence of receptors for C1q on effector cells modulates its activity, which may be antibody-dependent or independent. Macrophages are the primary source of C1q, while anti-inflammatory drugs as well as cytokines differentially regulate expression of the mRNA as well as the protein. However, its ability to modulate the interaction of platelets with collagen and immune complexes suggests C1q influences homeostasis as well as other immune activities, and perhaps thrombotic complications resulting from immune injury. Defects in C1q-A, C1q-B and C1q-C cause inactivation of the classical pathway, leading to a rare genetic disorder characterized by lupus-like symptoms.

Function:
C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes.

Subunit:
C1 is a calcium-dependent trimolecular complex of C1q, R and S in the molar ration of 1:2:2. C1q subcomponent is composed of nine subunits, six of which are disulfide-linked dimers of the A and B chains, and three of which are disulfide-linked dimers of the C chain.

Subcellular Location:
Secreted.

Post-translational modifications:
O-linked glycans consist of Glc-Gal disaccharides bound to the oxygen atom of post-translationally added hydroxyl groups.

DISEASE:
Defects in C1QA are the cause of complement component C1qA deficiency (C1QAD) [MIM:120550]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.

Similarity:
Contains 1 C1q domain.
Contains 1 collagen-like domain.

Gene ID:
712

C1Q,补体C1qα链多肽抗体Database links:
Entrez Gene: 712 Human
Entrez Gene: 713 Human
Entrez Gene: 714 Human
Entrez Gene: 12259 Mouse
Entrez Gene: 12260 Mouse
Entrez Gene: 12262 Mouse
Entrez Gene: 29687 Rat
Entrez Gene: 298566 Rat
Entrez Gene: 362634 Rat
Omim: 120550 Human
Omim: 120570 Human
Omim: 120575 Human
SwissProt: P02745 Human
SwissProt: P02746 Human
SwissProt: P02747 Human
SwissProt: P14106 Mouse
SwissProt: P98086 Mouse
SwissProt: Q02105 Mouse
SwissProt: P31720 Rat
SwissProt: P31721 Rat
SwissProt: P31722 Rat
Unigene: 632379 Human
Unigene: 439957 Mouse
Unigene: 105647 Rat

C1Q,补体C1qα链多肽抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.