更新于：2024-09-19

Complement Component 5 Deficiency

补充成分5缺陷

更新于：2024-09-19

基本信息

别名

C5 DEFICIENCY、C5 Deficiency、C5D

+ [9]

简介

A rare genetic disorder with an autosomal recessive pattern of inheritance. It is caused by the ineffective or decreased biosynthesis of the fifth complement component, C5. C5 deficiency may also be acquired acutely post-infection. If C5 is adequately synthesized, its rapid depletion may result in a functional deficiency. Clinical signs of the inherited deficiency present within the second decade of life and are consistent with the signs of recurrent systemic infection. Deficiency of serum C5 and its major cleavage product, C5b, a component of the membrane attack complex, increases susceptibility to Neisserial infections.

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100 项与补充成分5缺陷相关的临床结果

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100 项与补充成分5缺陷相关的转化医学

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0 项与补充成分5缺陷相关的专利（医药）

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188

项与补充成分5缺陷相关的文献（医药）

2024-02-05·European journal of immunology

Fcgr2b and Fcgr3 are the major genetic factors for cartilage antibody-induced arthritis, overriding the effect of Hc encoding complement C5.

Article

作者: Zhongwei Xu ; Àlex Moreno-Giró ; Danxia Zhao ; Alexander Krämer ; Rajan Kumar Pandey ; Bingze Xu ; Susanna L Lundström ; Rikard Holmdahl

Like rheumatoid arthritis (RA) in humans, collagen-induced arthritis (CIA) in mice is associated with not only MHC class II genetic polymorphism but also, to some extent, with other loci including genes encoding Fc gamma receptors (FCGRs) and complement C5. In this study, we used a cartilage antibody-induced arthritis (CAIA) model in which arthritis develops within a 12-h timeframe, to determine the relative importance of FCGRs and C5 (Hc). In CAIA, inhibiting or deleting FCGR3 substantially hindered arthritis development, underscoring the crucial role of this receptor. Blocking FCGR3 also reduced the levels of FCGR4, and vice versa. When employing an IgG1 arthritogenic cocktail that exclusively interacts with FCGR2B and FCGR3, joint inflammation was promptly initiated in Fcgr2b^-- mice but not in Fcgr3^-- mice, suggesting that FCGR3 is sufficient for CAIA development. Regarding complement activation, Fcgr2b⁺⁺ .Hc^** mice with C5 mutated were fully resistant to CAIA, whereas Fcgr2b^-- .Hc^** mice developed arthritis rapidly. We conclude that FCGR3 is essential and sufficient for CAIA development, particularly when induced by IgG1 antibodies. The human ortholog of mouse FCGR3, FCGR2A, may be associated with RA pathogenesis. FCGR2B deficiency allows for rapid arthritis progression and overrides the resistance conferred by C5 deficiency.

2023-09-01·Neurosurgical focus

Does hinge sidedness influence laterality of C5 palsy after expansile open-door cervical laminoplasty?

Article

作者: David J Levi ; G Damian Brusko ; Allan D Levi ; Michael Y Wang

OBJECTIVE:

Cervical expansile open-door laminoplasties (EOLPs) have an open side and a hinge side, with the open side being bridged by grafts or miniplates. The authors explored the possibility that the open-door side might have a greater incidence of C5 palsy due to a greater stretch of the ipsilateral C5 nerve root.

METHODS:

This study was a retrospective review of prospectively collected data over a period of 25 years specifically assessing surgical complications. Included were patients who underwent EOLP for myelopathy, radiculopathy, or mild central cord injuries (American Spinal Injury Association Impairment Scale [AIS] grade D). Exclusion criteria included preexisting C5 weakness; patients with AIS grade A, B, or C injury; and added instrumentation or additional surgical procedures. Patients were monitored postoperatively for C5 palsy or any other complications. A comparison group included patients who underwent cervical laminectomy and fusion (CLF).

RESULTS:

A total of 327 laminoplasties were collected, and 31 patients were excluded because of severe spinal cord injury (AIS grades A-C), 3 for preoperative C5 weakness, and 21 for instrumentation or additional surgical procedures. Thus, 272 patients were analyzed with a mean age of 59.9 years (range 22-88 years). Diagnoses at presentation were cervical myelopathy (84.1%), central cord syndrome (7.2%), cervical myeloradiculopathy (3.4%), ossification of the posterior longitudinal ligament (1.9%), and other (3.4%). The most common complications were C5 palsy (n = 7, 2.6%) and wound infection (n = 7, 2.6%). Of the 7 cases of postoperative C5 palsies in this study, 6 occurred on the side of the open door. Of the C5 palsies, 2 were mild, 3 were moderate, and 2 were severe. Two of the 7 C5 palsies had a delayed (> 24 hours) onset. The C5 palsy incidence after CLF was 2.7% with no side preference.

CONCLUSIONS:

C5 palsy after cervical decompression for myelopathy is a known occurrence, with a rate of 2.6% in the current study. The authors found that C5 palsies more commonly occur on the open side of the laminoplasty. This could be due to a greater manipulation of the nerve root on the side of the open door or greater stretch of the C5 root on the open-door side. If clinical symptoms and anatomical stenosis are symmetric, the authors recommend creating the laminoplasty hinge on the patient's dominant side to minimize potential loss of dominant proximal arm function.

2023-01-01·Neurotrauma reports

Complement Component 5 (C5) Deficiency Improves Cognitive Outcome After Traumatic Brain Injury and Enhances Treatment Effects of Complement Inhibitors C1-Inh and CR2-Crry in a Mouse Model.

Article

作者: Min Chen ; Stephen R Edwards ; Dhiraj Maskey ; Trent M Woodruff ; Stephen Tomlinson ; David Reutens

A potent effector of innate immunity, the complement system contributes significantly to the pathophysiology of traumatic brain injury (TBI). This study investigated the role of the complement cascade in neurobehavioral outcomes and neuropathology after TBI. Agents acting at different levels of the complement system, including 1) C1 esterase inhibitor (C1-Inh), 2) CR2-Crry, an inhibitor of all pathways acting at C3, and 3) the selective C5aR1 antagonist, PMX205, were administered at 1 h post-TBI. Their effects were evaluated on motor function using the rotarod apparatus, cognitive function using the active place avoidance (APA) task, and brain lesion size at a chronic stage after controlled cortical impact injury in C5-sufficient (C5^+/+) and C5-deficient (C5^-/-) CD1 mice. In post-TBI C5^+/+ mice, rotarod performance was improved by CR2-Crry, APA performance was improved by CR2-Crry and PMX205, and brain lesion size was reduced by PMX205. After TBI, C5^-/- mice performed better in the APA task compared with C5^+/+ mice. C5 deficiency enhanced the effect of C1-Inh on motor function and brain damage and the effect of CR2-Crry on brain damage after TBI. Our findings support critical roles for C3 in motor deficits, the C3/C5/C5aR1 axis in cognitive deficits, and C5aR1 signaling in brain damage after TBI. Findings suggest the combination of C5 inhibition with C1-Inh and CR2-Crry as potential therapeutic strategies in TBI.

项与补充成分5缺陷相关的新闻（医药）

2023-07-06

·PipelineReview

Complement C5 Inhibitors & C5 Receptor Antagonists: a Competitor Analysis

BARCELONA, Spain I July 6, 2023 I La Merie Publishing released its newest product reviewing the pipeline of complement C5 inhibitors and C5 receptor antagonists: Competitor Analysis: Complement C5 Inhibitors & C5 Receptor Antagonists This Competitive Intelligence report about Complement C5 Inhibitors & C5 Receptor Antagonists provides a competitor evaluation in the field of mainly recombinant antibodies, but also proteins, peptides, RNA and small molecules acting as inibitors of complement C5 or antagonists of the C5 receptor as of July 2023. With annual sales of more than US$ 5.7 bln in 2022, first and second generation C5 inhibitor antibodies Soliris and Ultomiris from AstraZeneca (acquired Alexion Pharmaceuticals) have set the stage for next generation biosuperiors as well as biosimilars of a commercially successful and clinically effective treatment of complement C5 mediated diseases. New treatment modalities in development aim at improving the mode and frequency of administration as well as the scope of indications. Players in the field not only include small and medium biotech, but also Big Pharmas This product consists of a competitor description in a tabular format provided as a pdf document and an one-month online access to La Merie Publishing’s online database for this product (report code: LMCA0012) (prerequisite: access to internet). Both deliverables (pdf and database credentials) are sent by e-mail within one day, usually within one hour. The pdf report includes a compilation of currently active projects in research and development of inhibitors of complement C5 or antagonists of the C5a receptor. In addition, the pdf report lists company-specific R&D pipelines of C5 inhibitors & C5R antagonists. Competitor projects are listed in a tabular format providing information on: About Competitor Analysis reports: Competitor Analysis reports deliver NO-FRILLS, but concise information about the pipeline of R&D projects for targets, diseases, technologies and companies at low prices. The information is provided in a tabular format and fully referenced. The LMCA0012 report database provides access to the history of each project with links to the source of information (press release, homepage, abstracts, presentations, annual reports etc). Furthermore, the database allows export of data into a Word or Excel document. The report “ Competitor Analysis: Complement C5 Inhibitors & C5 Receptor Antagonists ” can be acquired at La Merie Publishing’s online store: https://lamerie.com/report/competitor-analysis-complement-c5-inhibitors-c5-receptor-antagonists/ About La Merie Publishing La Merie Publishing is an independent business information provider for the biotechnology and pharmaceutical industry. La Merie Publishing prepares brief and full reports. La Merie Publishing products can be purchased in the online store www.lamerie.com and from selected Resellers. La Merie Publishing offers the service of custom report preparation for corporate clients, including, but not limited to, pipeline analysis reports, drug profiles and any other competitive intelligence elaboration of interest. SOURCE: La Merie Publishing