UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. C3糸球体腎症：デンスデポジット病およびC3糸球体腎炎 c3 glomerulopathies dense deposit disease and c3 glomerulonephritis
2. 膜性増殖性糸球体腎炎の臨床像、分類、および原因 clinical presentation classification and causes of membranoproliferative glomerulonephritis
3. Acquired deficiencies of the complement system
4. C3糸球体症の移植後再発 recurrence of c3 glomerulopathies after transplantation
5. C4 glomerulopathy

English Journal

Insights into the effects of complement Factor H on the assembly and decay of the alternative pathway C3 proconvertase and C3 convertase.

Bettoni S1, Bresin E1, Remuzzi G1, Noris M2, Donadelli R1.
The Journal of biological chemistry.J Biol Chem.2016 Feb 22. pii: jbc.M115.693119. [Epub ahead of print]
C3b and factor B (FB) form the C3 proconvertase (C3bB), which is cleaved by factor D (FD) to C3 convertase (C3bBb). Old papers indicated that the complement AP regulator factor H (FH) competes with FB for C3b binding, however FH capability to prevent C3bB assembly has not been formally investigated.
PMID 26903516

Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy.

Corvillo F1,2, García-Morato MB1, Nozal P1,2, Garrido S1,2, Tortajada A3, de Córdoba SR3, López-Trascasa M1,2.
Clinical and experimental immunology.Clin Exp Immunol.2015 Dec 13. doi: 10.1111/cei.12754. [Epub ahead of print]
Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non-self surfaces. Although P deficiencies are long time known to be associated with N
PMID 26660535

Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression.

Häffner K1, Michelfelder S2, Pohl M2.
Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2015 Nov;30(11):1951-9. doi: 10.1007/s00467-015-3111-9. Epub 2015 May 19.
BACKGROUND: C3 glomerulopathies (C3G) are characterized by uncontrolled activation of the alternative pathway of complement. In most patients these diseases progress towards end-stage renal disease, and the risk of recurrence after renal transplantation is high. In the majority of patients, only ant
PMID 25986912

Japanese Journal

Atypical Postinfectious Glomerulonephritis Is Associated with Abnormalities in the Alternative Pathway of Complement

, , , , , , ,
日本小児腎臓病学会雑誌 26(2), 305-306, 2014
… C3Nef）のいずれか，あるいは両方を認めた。 …
NAID 130004836709

A case of dense deposit disease associated with a group A streptococcal infection without the involvement of C3NeF or complement factor H deficiency

Suga Kenichi,Kondo Shuji,Matsuura Sato,Kinoshita Yukiko,Kitano Etsuko,Hatanaka Michiyo,Kitamura Hajime,Hidaka Yoshihiko,Oda Takashi,Kagami Shoji
PEDIATRIC NEPHROLOGY 25(8), 1547-1550, 2010
NAID 120004738149

家族性低Ｃ３血症の臨床学的検討とその発症機序に関する検討

香坂隆夫,池谷健,小林登
日本小児腎臓病学会雑誌 9(1), 11-15, 1996
… これらの患者にしばしばC3NeF活性や，補体のbreakdown productsの存在が認められることより，補体の活性化による低下という面からの検討を行った。 … Spitzerらは，C3NeFについてはidiotype検体の出現があり，これがC3NeFは産生亢進，抑制を認節している可能性について報告4)5)している。 …
NAID 130000127731

Related Pictures

Facteur Néphritique C3 ; c3nef; facteur c3 nephritic factor c3nef c3nef stabilizes lisis de células glomerulares por C3Nef C3Nef Puede con baja Properdina Puede con C3NeF = C3 nephritic factor found in the C3NeF