淋巴细胞底膜的结构完整性决定了脏中拉米宁的跨组织沉积
Kohei Omachi1, Meei-Hua Lin2, Pongpratch Puapatanakul2
1Division of Nephrology, Washington University School of Medicine, St. Louis, MO 63110, USA; Laboratory for Tissue Microenvironment, RIKEN Center for Biosystems Dynamics Research (BDR), Kobe 650-0047, Japan.
Cell reports
|February 19, 2026
概括
脏球底膜 (GBM) 中的异位拉米因-α2来源于循环,而不是局部细胞. 这揭示了一种非细胞自主机制,调节哺乳动物的底层膜组成.
科学领域:
- 生物化学 生物化学
- 细胞生物学 细胞生物学
- 腎臟病學 (nephrology) 是一種醫學專業.
背景情况:
- 底层膜 (BMs) 是组织结构和功能的关键细胞外矩阵 (ECMs).
- 据认为,哺乳动物的ECM主要由局部细胞产生.
- 阿尔波特综合征涉及GBM缺陷和未知的拉米林-α2来源.
研究的目的:
- 为了调查异位拉米林-α2在球底膜 (GBM) 中的起源.
- 探索循环蛋白质对哺乳动物的BM组成的贡献.
主要方法:
- 在转基因模型中利用CRISPR-Cas9基因编辑.
- 分析了GBM和介质层中的蛋白质沉积.
主要成果:
- 在GBM中异胎性拉米林-α2源于循环.
- 即使在健康的脏中,循环来源也会对中层胺-α2产生贡献.
- GBM 完整性影响循环蛋白质的结合.
结论:
- 哺乳动物的BM组成是由跨组织机制调节的.
- 这挑战了对ECM生产的传统观点.
- 确定了一种用于BM调节的新型非细胞自主途径.
相关概念视频
Basal Lamina are the Specialized Form of ECM
The basal lamina is a thin extracellular layer that lies underneath the cells and separates them from other tissues. The three layers of the basal lamina are lamina lucida, lamina densa and lamina reticularis. The basal lamina, a mixture of glycoproteins and collagen, provides an attachment site for the epithelium, separating it from underlying connective tissue. The framework of basal lamina has other essential proteins such as laminins mesh, perlecan, entactin, and type IV collagen.
Proteins...
Proteins...
Laminins are the Adhesive Proteins of Basal Lamina
Laminins are heterotrimeric proteins with high molecular mass found in the extracellular matrix. Each laminin molecule is composed of three chains, viz. alpha, beta, and gamma, coded by five, four, and three paralogous genes, respectively. Laminins are categories based on the compositions of the three chains.
In humans, the five forms of alpha chains are LAMA 1, LAMA 2, LAMA 3, LAMA 4, and LAMA 5. The four forms of beta chains are LAMB 1, LAMB 2, LAMB 3, and LAMB 4. The three forms of gamma...
In humans, the five forms of alpha chains are LAMA 1, LAMA 2, LAMA 3, LAMA 4, and LAMA 5. The four forms of beta chains are LAMB 1, LAMB 2, LAMB 3, and LAMB 4. The three forms of gamma...
Type IV Collagen of Basal Lamina
Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
A type IV collagen molecule has six alpha chains which can exist in...
A type IV collagen molecule has six alpha chains which can exist in...
Integrins
Animal and protozoan cells do not have cell walls to help maintain shape and provide structural stability. Instead, these eukaryotic cells secrete a sticky mass of carbohydrates and proteins into the spaces between adjacent cells. This network of proteins and molecules is called an extracellular matrix or ECM.
Some ECM proteins assemble into a basement membrane to which the remaining components adhere. Proteoglycans typically form the bulk of the ECM while fibrous proteins, like collagen,...
Some ECM proteins assemble into a basement membrane to which the remaining components adhere. Proteoglycans typically form the bulk of the ECM while fibrous proteins, like collagen,...
Renal Corpuscle
The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
Glomerular Filtration
The filtration membrane in the renal system is a highly specialized structure essential for filtering blood. It consists of glomerular capillaries and podocytes, forming a selective barrier that permits the passage of water and small solutes while restricting most plasma proteins and blood cells.
Components of the Filtration Membrane
The filtration process involves three key layers: the glomerular endothelial cells, the basement membrane, and the podocyte-formed filtration slits.
Components of the Filtration Membrane
The filtration process involves three key layers: the glomerular endothelial cells, the basement membrane, and the podocyte-formed filtration slits.


