WordNet

the lowermost portion of a structure partly or wholly below ground level; often used for storage (同)cellar
the ground floor facade or interior in Renaissance architecture
a thin pliable sheet of material
a pliable sheet of tissue that covers or lines or connects the organs or cells of animals or plants (同)tissue layer
of or relating to glomeruli

PrepTutorEJDIC

地階,地下室
(生物の)膜,皮膜

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/12/22 04:40:56」(JST)

wiki en

The glomerular basement membrane (GBM) of the kidney is the basal lamina layer of the glomerulus. The glomerular capillary endothelial cells, the GBM and the filtration slits between the podocytes perform the filtration function of the glomerulus, separating the blood in the capillaries from the filtrate that forms in Bowman's capsule.^[1] The GBM is a fusion of the endothelial cell and podocyte basal laminas.^[2]

Layers

The GBM contains three layers: ^[3]

Layer	Location	Composition	Function
lamina rara externa	adjacent to podocyte processes	heparan sulfate	blocks by charge
lamina densa	dark central zone	type 4 collagen and laminin	blocks by size (Molecular Weight > 5800kDa)
lamina rara interna	adjacent to endothelial cells	heparan sulfate	blocks by charge

The glomerular membrane consists of mesangial cells, modified pericytes that in other parts of the body separate capillaries from each other. The podocytes adjoining them have filtration slits of diameter 25 nm that are formed by the pseudopodia arising from them. The filtration slits are covered by a diaphragm that includes the transmembrane protein nephrin.

Pathology

Micrograph showing GBM spike formation in membranous nephropathy. Jones stain.

Goodpasture's syndrome is also known as "anti-glomerular basement membrane disease". Capillaries become inflamed as a result of damage to the basement membrane by antibodies to alpha 3 NC1 domain of type IV collagen.
Nephrotic syndrome is a change in the structure of the glomerular filtration mechanism usually in the glomerular basement membrane. Some symptoms include proteinuria, hypoalbuminaemia, oedema, and hyperlipidemia.
Diabetic glomerulosclerosis is a thickening of the basement membrane, which can become up to 4-5 times thicker than normal. Can be caused by insulin deficiency or resultant hyperglycemia.
Alport syndrome is a X-linked hereditary nephritis caused by mutations in type IV collagen, leading to a split lamina densa of the glomerular basement membrane. It also affects the eye and inner ear.

Additional images

Renal corpuscle. GBM is #1.

References

^ Jarad, G.; Miner, J. H. (2009). "Update on the glomerular filtration barrier". Current opinion in nephrology and hypertension 18 (3): 226–232. doi:10.1097/mnh.0b013e3283296044. PMC 2895306. PMID 19374010. edit
^ Physiology at MCG 7/7ch04/7ch04p07 - "Basement Membrane"
^ JCI - New articles published

External links

glomerular basement membrane at the US National Library of Medicine Medical Subject Headings (MeSH)

"The GBM and its disorders" at dmed.ed.ac.uk

UpToDate Contents

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1. 抗GBM抗体（グッドパスチャー）病の病因および診断 pathogenesis and diagnosis of anti gbm antibody goodpastures disease
2. 抗GBM抗体（グッドパスチャー）病の治療 treatment of anti gbm antibody goodpastures disease
3. 抗GBM抗体病：移植後の再発 anti gbm antibody disease recurrence after transplantation
4. 糸球体の免疫障害の機序 mechanisms of immune injury of the glomerulus
5. 糸球体半月体形成の機序 mechanisms of glomerular crescent formation

English Journal

A mouse Col4a4 mutation causing Alport glomerulosclerosis with abnormal collagen α3α4α5(IV) trimers.

Korstanje R1, Caputo CR1, Doty RA1, Cook SA1, Bronson RT1, Davisson MT1, Miner JH2.Author information 1The Jackson Laboratory, Bar Harbor, Maine, USA.2Renal Division, Washington University School of Medicine, St Louis, Missouri, USA.AbstractA spontaneous mutation termed bilateral wasting kidneys (bwk) was identified in a colony of NONcNZO recombinant inbred mice. These mice exhibit a rapid increase of urinary albumin at an early age associated with glomerulosclerosis, interstitial nephritis, and tubular atrophy. The mutation was mapped to a location on chromosome 1 containing the Col4a3 and Col4a4 genes, for which mutations in the human orthologs cause the hereditary nephritis Alport syndrome. DNA sequencing identified a G-to-A mutation in the conserved GT splice donor of Col4a4 intron 30, resulting in skipping of exon 30 but maintaining the mRNA reading frame. Protein analyses showed that mutant collagen α3α4α5(IV) trimers were secreted and incorporated into the glomerular basement membrane (GBM), but levels were low, and GBM lesions typical of Alport syndrome were observed. Moving the mutation into the more renal damage-prone DBA/2J and 129S1/SvImJ backgrounds revealed differences in albuminuria and its rate of increase, suggesting an interaction between the Col4a4 mutation and modifier genes. This novel mouse model of Alport syndrome is the only one shown to accumulate abnormal collagen α3α4α5(IV) in the GBM, as also found in a subset of Alport patients. These mice will be valuable for testing potential therapies, for understanding abnormal collagen IV structure and assembly, and for gaining better insights into the mechanisms leading to Alport syndrome, and to the variability in the age of onset and associated phenotypes.Kidney International advance online publication, 12 February 2014; doi:10.1038/ki.2013.493.
Kidney international.Kidney Int.2014 Feb 12. doi: 10.1038/ki.2013.493. [Epub ahead of print]
A spontaneous mutation termed bilateral wasting kidneys (bwk) was identified in a colony of NONcNZO recombinant inbred mice. These mice exhibit a rapid increase of urinary albumin at an early age associated with glomerulosclerosis, interstitial nephritis, and tubular atrophy. The mutation was mapped
PMID 24522496

Effect of a combined treatment with erythropoietin and melatonin on renal ischemia reperfusion injury in male rats.

Ahmadiasl N, Banaei S, Alihemati A, Baradaran B, Azimian E.Author information Drug Applied Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.AbstractBACKGROUND: Renal ischemia reperfusion (IR) is an important cause of renal dysfunction. It contributes to the development of acute renal failure. Oxidative damage from reactive oxygen species is considered to be the principal component involved in the pathophysiological tissue alterations observed during IR. The purpose of this study was to evaluate the effect of a combined treatment with erythropoietin (EPO) plus melatonin (MEL), which are known anti-inflammatory and antioxidant agents, in IR-induced renal injury in rats.
Clinical and experimental nephrology.Clin Exp Nephrol.2014 Feb 4. [Epub ahead of print]
BACKGROUND: Renal ischemia reperfusion (IR) is an important cause of renal dysfunction. It contributes to the development of acute renal failure. Oxidative damage from reactive oxygen species is considered to be the principal component involved in the pathophysiological tissue alterations observed d
PMID 24493464

[C3 glomerulopathy.]

Chauvet S1, Servais A2, Frémeaux-Bacchi V3.Author information 1Service de néphrologie, hôpital Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France. Electronic address: sophie.chauvet@egp.aphp.fr.2Service de néphrologie, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France.3Laboratoire d'immunologie, hôpital Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France.AbstractC3 glomerulopathy is an heterogeneous group of glomerular diseases associated with acquired or genetic abnormalities of complement alternative pathway (AP) components. It is characterized by predominant C3 deposits in the mesangium and along the glomerular basement membrane (GBM). Presenting features comprise proteinuria (sometimes with nephritic syndrome), haematuria, hypertension and renal failure. C3 glomerulopathy have a poor renal prognosis with progression to end stage renal disease (ESRD) in 50% of cases during the first decade after initial presentation. Moreover, C3 deposits recur in most of cases after renal transplantation. Patients frequently have low serum C3 level attributed to the activation of the alternative pathway of complement. Animal models have confirmed the role of excessive C3 activation in the pathogenesis of C3 glomerulopathy. To date, the optimal treatment remains unknown. It is currently based on the use of angiotensin-converting-enzyme inhibitors (ACEI) and angiotensin II-receptor blockers (ARB), sometimes associated with immunosuppressive therapy. Blockade of C5a release with eculizumab, a monoclonal anti-C5 antibody, may be of particular interest in the treatment of C3G.
Nephrologie & therapeutique.Nephrol Ther.2014 Feb 4. pii: S1769-7255(13)00611-1. doi: 10.1016/j.nephro.2013.09.007. [Epub ahead of print]
C3 glomerulopathy is an heterogeneous group of glomerular diseases associated with acquired or genetic abnormalities of complement alternative pathway (AP) components. It is characterized by predominant C3 deposits in the mesangium and along the glomerular basement membrane (GBM). Presenting feature
PMID 24508002

Japanese Journal

症例ステロイドが奏効したMPO-ANCA陽性のGoodpasture症候群の1例

安部光洋,須田明,吉田正 [他]
日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 2(1), 18-23, 2013-01-10
NAID 40019560206

Unchanged Distribution Density of Anionic Sites on the Glomerular Wall in Rats with Active Heymann Nephritis

Shimoi Akihito,Hatakeyama Hirofumi,Miyoshi Seiji [他]
Journal of toxicologic pathology 26(1), 11-17, 2013
NAID 40019696615

補体第4成分(C4)低下を伴うループス腎炎様の組織像を呈した1例

伊達慶一,石原正行,菊地広朗,松本学,成瀬桂史,浜田義文,藤枝幹也
Nihon Shoni Jinzobyo Gakkai Zasshi 26(1), 122-125, 2013
　症例は14歳男児。学校検尿で血尿蛋白尿を指摘され，精査で補体第4成分(C4)値の持続的低下を認めた。家族歴に特記事項はなく，身体所見も異常を認めなかった。C4値以外の血液検査所見，画像検査は異常を認めなかった。腎生検所見で多彩な病変を認め，ループス腎炎様の所見であった。ステロイドの投与により尿所見は改善し，外来経過観察中である。C4の低下は一般人口においてもみられるが，C4の完全欠損は全身性エリ …
NAID 130003384551

Related Pictures

★リンクテーブル★

リンク元	「糸球体」「ボーマン嚢」「GBM」「糸球体基底膜」
拡張検索	「anti-glomerular basement membrane disease」「anti-glomerular basement-membrane antibody disease」「thin glomerular basement membrane disease」
関連記事	「glomerular」「basement」「membrane」

「糸球体」

Glomerular basement membrane
	Glomerulus. (Diagram in French, but "Membrane basale glomerulaire et ses podocytes" labeled near center.)
	Scheme of filtration barrier (blood-urine) in the kidney. A. The endothelial cells of the glomerulus; 1. pore (fenestra).; B. Glomerular basement membrane: 1. lamina rara interna 2. lamina densa 3. lamina rara externa; C. Podocytes: 1. enzymatic and structural protein 2. filtration slit 3. diaphragm
Identifiers
MeSH	glomerular+basement+membrane
Dorlands; /Elsevier	m_08/12522684
	Anatomical terminology

　　[★]

英: glomerulus (Z)
関: ボウマン嚢、腎小体、腎臓、輸出細動脈#調節

腎小体 = 糸球体 + ボーマン嚢

概念

1個の腎臓に100万個存在。
房状の毛細血管からなる
糸球体の毛細血管は有窓型毛細血管である

糸球体の微細構造 (SP.785)

血管内皮細胞
基底膜
上皮細胞間隙

詳細には

糸球体内皮細胞 endothelium
基底膜 basement membrane, glomerular basement membrane
糸球体上皮細胞 visceral epithelium, podocyte
ボウマン嚢の壁側板 parietal epithelium

血流の調節 (HIM.1742)

輸入細動脈：autonomous vasoreactive reflex in afferent arteriole, tubuloglomerular feedback

緻密斑でのCl^-の低下 = 原尿流速が早い →　腎灌流量の低下と解釈

腎灌流圧↑→輸入細動脈の平滑筋収縮
腎灌流圧↓→輸入細動脈の平滑筋弛緩

緻密斑はNaClの再吸収にとともにATP、(アデノシン)を細胞外に放出。細胞外のecto-5'-nucleotidaseがATPからアデノシンを産生。アデノシンが輸入細動脈のvasoconstrictorとして作用

loop diureticsは緻密斑でのNaClの再吸収を妨げるので、尿細管糸球体フィードバックを阻害→糸球体濾過量は高レベルに保たれる
アンジオテンシンIIと活性酸素種は尿細管糸球体フィードバックを増強　→　輸入細動脈収縮　→　糸球体濾過量低下
NOは尿細管糸球体フィードバックを減弱　→　輸入細動脈弛緩　→　糸球体濾過量上昇

輸出細動脈：angiotensin II-mediated casoconstriction of the efferent arteiole

アンジオテンシンII→輸出細動脈収縮

輸入細動脈顆粒細胞により放出されたレニンは結局アンジオテンシンIIを産生し、輸出細動脈に作用してこれを収縮させる。

臨床関連

糸球体疾患

「ボーマン嚢」

　　[★]

英: Bowman's capsule (Z), Bowman capsule
ラ: capsula glomeruli
同: ボウマン嚢、糸球体嚢糸球体包 glomerular capsule
関: 糸球体、腎臓、腎小体、ネフロン

[show details]

ボーマン嚢 : 約 5,230 件
ボウマン嚢 : 約 4,780 件

糸球体内皮細胞 endothelium
基底膜 basement membrane, glomerular basement membrane
糸球体上皮細胞 visceral epithelium, podocyte
ボウマン嚢の壁側板 parietal epithelium

「GBM」

　　[★]

「糸球体基底膜」

　　[★]

英: glomerular basement membrane, GBM
関: 糸球体

「anti-glomerular basement membrane disease」

　　[★] 抗糸球体基底膜抗体病

「anti-glomerular basement-membrane antibody disease」