A case report of complement C4B deficiency in a patient with steroid and IVIG-refractory anti-NMDA receptor encephalitis. [PDF]
Background Complement C4A or C4B deficiency has never been reported in autoantibody-associated encephalitides patient. Here we present a case of anti-N-methyl- D-aspartate (NMDA) receptor encephalitis associated with homozygous C4B deficiency, who did ...
Chua GT+5 more
europepmc +3 more sources
Real-time PCR quantification of human complement C4A and C4B genes [PDF]
AbstractBackgroundThe fourth component of human complement (C4), an essential factor of the innate immunity, is represented as two isoforms (C4A and C4B) in the genome. Although these genes differ only in 5 nucleotides, the encoded C4A and C4B proteins are functionally different.
Zsolt Ronai+5 more
doaj +7 more sources
Serum complement C4b, fibronectin, and prolidase are associated with the pathological changes of pulmonary tuberculosis [PDF]
AbstractBackgroundMycobacterium tuberculosisinfection can activate the immune system, leading to characteristic pathological changes such as inflammatory granuloma, caseous necrosis, and cavity formation.MethodsClinical data of 187 cases of pulmonary tuberculosis (PTB) were analyzed using statistical methods, while serum levels of complement C4b (C4b),
Xiuyun Yang+12 more
europepmc +5 more sources
Clinical features of patients with homozygous complement C4A or C4B deficiency
Homozygous deficiencies of complement C4A or C4B are detected in 1-10% of populations. In genome-wide association studies C4 deficiencies are missed because the genetic variation of C4 is complex. There are no studies where the clinical presentation of these patients is analyzed.
Inka Liesmaa+4 more
doaj +7 more sources
Human Pentraxin 3 Binds to the Complement Regulator C4b-Binding Protein [PDF]
The long pentraxin 3 (PTX3) is a soluble recognition molecule with multiple functions including innate immune defense against certain microbes and the clearance of apoptotic cells. PTX3 interacts with recognition molecules of the classical and lectin complement pathways and thus initiates complement activation.
Anne Braunschweig, Mihály Józsi
doaj +6 more sources
The Pneumococcal Surface Proteins PspA and PspC Sequester Host C4-Binding Protein To Inactivate Complement C4b on the Bacterial Surface. [PDF]
Complement is a critical component of antimicrobial immunity. Various complement regulatory proteins prevent host cells from being attacked. ABSTRACT Complement is a critical component of antimicrobial immunity.
Haleem KS+7 more
europepmc +2 more sources
Objective Systemic lupus erythematosus (SLE) features high frequency of cardiovascular disease (CVD) and fluctuating complement levels. The clinical trial Atherosclerosis Prevention in Pediatric Lupus Erythematosus (APPLE) aimed to evaluate whether ...
Evan Mulvihill+12 more
doaj +2 more sources
Substitution of a single amino acid (aspartic acid for histidine) converts the functional activity of human complement C4B to C4A. [PDF]
The C4B isotype of the fourth component of human complement (C4) displays 3- to 4-fold greater hemolytic activity than does its other isotype C4A.
M. Carroll+4 more
semanticscholar +2 more sources
C4b-binding protein (C4BP) is a large complex assembly of eight subunits that functions as an inhibitor of the complement cascade. A portion of the C4BP in serum exists as a complex with protein S. This study demonstrated that another protein, serum amyloid P component (SAP), also formed a calcium-dependent complex with C4BP.
Gary L. Nelsestuen+2 more
openaire +4 more sources
Chimeric Proteins Containing MAP-1 and Functional Domains of C4b-Binding Protein Reveal Strong Complement Inhibitory Capacities [PDF]
The complement system is a tightly regulated network of proteins involved in defense against pathogens, inflammatory processes, and coordination of the innate and adaptive immune responses.
C. Hertz+6 more
semanticscholar +2 more sources