相关实验视频
Updated: Jan 10, 2026
01:24
Nephrotic Syndrome I : Introduction
Published on: June 19, 2025
476
在分泌蛋白质pepsinogen中生成 lysosomal酶准信号
T J Baranski1, P L Faust, S Kornfeld
1Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri 63110.
Cell
|October 19, 1990
概括
研究人员确定了一种特定的蛋白质决定者在lysosomal酶上,这对于曼诺6酸盐形成至关重要. 这种由两个非连续序列的甲素D形成的决定因素,对于酶向和酸化至关重要.
科学领域:
- 细胞生物学 细胞生物学
- 生物化学 生物化学
- 分子生物学分子生物学
背景情况:
- 溶解体酶需要特定的修改,以适当地准细胞.
- 曼诺6酸盐 (M6P) 残留物对于将 lysosomal 酶引导到 lysosomes 的过程至关重要.
- 酶UDP-GlcNAc:lysosomal酶N-乙糖胺-1-光转移酶通过识别蛋白质决定物来启动M6P的形成.
研究的目的:
- 为了确定UDP-GlcNAc识别的特定蛋白质决定因素:lysosomal酶N-乙糖胺-1-光转移酶.
- 阐明溶酶酶识别和M6P标记的结构基础.
主要方法:
- 在 lysosomal 酶 cathepsin D 和分泌蛋白 pepsinogen 之间构建化学分子.
- 在Xenopus卵细胞中表达仿真分子的表达.
- 对寡糖类酸化模式的分析.
主要成果:
- 甲素D寡糖化合物被有效酸化,而素原寡糖化合物没有.
- 将两个非连续的甲素D序列 (Lys203和残留物265-292) 替换为甘素素,导致酸化.
- 这两个序列在蛋白质表面的空间上相邻,形成一个识别域.
结论:
- 一种特定的蛋白质决定物,由空间上不同的氨基酸序列组成,介导了M6P形成的溶酶酶识别.
- 这个决定因素是由 cathepsin D 残留物 Lys203 和 265-292 形成的,它们在三维空间中聚集在一起.
- 其他地区可能有助于更广泛的识别标记 lysosomal 酶.
相关概念视频
Nephrotic Syndrome I : Introduction
476
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
476
Glomerular Filtration
4.7K
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.
4.7K
Renal Corpuscle
6.9K
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...
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...
6.9K
Confocal Fluorescence Microscopy
19.9K
Confocal microscopy is an advanced microscopic technique. The prime advantage of the confocal microscope over other microscopy techniques is its ability to block the out-of-focus light from the illuminated samples using pinholes. It is widely used with fluorescence optics to obtain high-resolution, sharp contrast images. Unlike optical microscopes, confocal microscopes use a focused beam of light laser to scan the entire sample surface at different z-planes. These microscopes are, therefore,...
19.9K
Nephrons
6.3K
The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
6.3K
Glomerular Filtration Rate and its Regulation
5.0K
The Glomerular Filtration Rate (GFR) is a measure of kidney function, reflecting the volume of filtrate formed per minute in the kidneys. On average, GFR is approximately 125 mL/min in males and 105 mL/min in females. Maintaining a relatively constant GFR is essential for the kidneys to effectively regulate body fluid homeostasis and maintain extracellular stability.
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
5.0K