系统性红斑狼疮
中文名称
通路描述
系统性红斑狼疮(SLE)是一种典型的自身免疫性疾病,其特征是产生针对自身抗原(如 DNA、核蛋白和某些细胞质成分)的特异性 IgG 自身抗体,并伴随多种临床表型。SLE 患者主要的病理发现是炎症、血管炎、免疫复合物沉积和血管病。由自身抗体和自身抗原组成的免疫复合物特异性沉积于肾小球,并通过激活补体或 FcγR 介导的中性粒细胞和巨噬细胞活化,引发全身性炎症反应。补体(C5)的激活会导致损伤,一方面通过形成膜攻击复合物(C5b-9),另一方面通过生成过敏毒素和细胞激活物 C5a。中性粒细胞和巨噬细胞通过释放氧化剂和蛋白酶导致组织损伤。
英文描述
Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and certain cytoplasmic components, in association with a diverse array of clinical manifestations. The primary pathological findings in patients with SLE are those of inflammation, vasculitis, immune complex deposition, and vasculopathy. Immune complexes comprising autoantibody and self-antigen is deposited particulary in the renal glomeruli and mediate a systemic inflammatory response by activating complement or via Fc{gamma}R-mediated neutrophil and macrophage activation. Activation of complement (C5) leads to injury both through formation of the membrane attack complex (C5b-9) or by generation of the anaphylatoxin and cell activator C5a. Neutrophils and macrophages cause tissue injury by the release of oxidants and proteases.
所含基因
141 个基因
ACTN1
ACTN4
C1QA
C1QB
C1QC
C1R
C1S
C2
C3
C4A
C4B
C5
C6
C7
C8A
C8B
C8G
C9
CD28
CD40
CD40LG
CD80
CD86
CTSG
ELANE
FCGR1A
FCGR2A
FCGR3A
FCGR3B
GRIN2A
GRIN2B
H2AB1
H2AB2
H2AB3
H2AC1
H2AC11
H2AC12
H2AC13
H2AC14
H2AC15
H2AC16
H2AC17
H2AC18
H2AC19
H2AC20
H2AC21
H2AC25
H2AC4
H2AC6
H2AC7
H2AC8
H2AJ
H2AL3
H2AX
H2AZ1
H2AZ2
H2BC1
H2BC10
H2BC11
H2BC12
H2BC12L
H2BC13
H2BC14
H2BC15
H2BC17
H2BC18
H2BC21
H2BC26
H2BC3
H2BC4
H2BC5
H2BC6
H2BC7
H2BC8
H2BC9
H2BK1
H2BW1
H2BW2
H3-3A
H3-3B
H3-4
H3-5
H3-7
H3C1
H3C10
H3C11
H3C12
H3C13
H3C14
H3C15
H3C2
H3C3
H3C4
H3C6
H3C7
H3C8
H3Y1
H3Y2
H4C1
H4C11
H4C12
H4C13
H4C14
H4C15
H4C16
H4C2
H4C3
H4C4
H4C5
H4C6
H4C7
H4C8
H4C9
HLA-DMA
HLA-DMB
HLA-DOA
HLA-DOB
HLA-DPA1
HLA-DPB1
HLA-DQA1
HLA-DQA2
HLA-DQB1
HLA-DQB2
HLA-DRA
HLA-DRB1
HLA-DRB3
HLA-DRB4
HLA-DRB5
IFNG
IL10
LOC102723407
LOC102724334
MACROH2A1
MACROH2A2
RO60
SNRPB
SNRPD1
SNRPD3
SSB
TNF
TRIM21