More detailed view of the process shown in Fig

Slides:

Advertisements

Similar presentations

Metabolism of ganglioside GM2 in GM2 activator-deficient fibroblasts

Advertisements

A: Population frequency histogram of urinary debrisoquine/4-hydroxydebrisoquine ratios after debrisoquine administration to 258 individuals in a healthy.

Genomic organization of the human cathepsin K gene

Simplified view of the cellular RNA and protein quality surveillance or control systems. Stop-codon introductions as well as splice-site variations, resulting.

Simplified view of the cellular RNA and protein quality surveillance or control systems. Stop-codon introductions as well as splice-site variations, resulting.

A, Diagnosis of clinical severity (mild, moderate, severe) of hemophilia is based on in vitro coagulant activity as shown. B, Distribution of severe and.

Spectrum of different types of human gene mutations logged in Human Gene Mutation Database as of September 13, 1998 ( Source: The.

Combinatorial interactions of cyclins and cyclin-dependent kinases (cdks) during the cell cycle. Progression from G0 through the restriction point in G1.

Physical map of a human chromosome

Patterns of genetic change in neuroblastomas

Morphologic appearance of EH skin. Shown is a semithin section (0

Distribution of diagnoses among the nearly 115,000 cases of lymphoid malignancy diagnosed in There are 5400 cases of acute lymphoid leukemia (ALL),

Distribution of diagnoses among the nearly 115,000 cases of lymphoid malignancy diagnosed in There are 5400 cases of acute lymphoid leukemia (ALL),

IX; formation of sphingolipids, sphingosine is synthesised from L-serine and palmitoyl-CoA. Source: Serine deficiency disorders, The Online Metabolic and.

Proposed model for the structure of ABC1 within the plasma membrane (modified from reference202 ). The two symmetric halves consisting of six membrane-spanning.

Illustration of the origin of tubular cysts

Structure of the A-subunit gene and identified mutations

Light micrographs comparing pycnodysostosis and normal bone

The lymphocyte markers that distinguish the various stages of B-cell differentiation are shown. Markers that are expressed on the cell surface are indicated.

Schematic representation of the DTDST protein

Radiographic appearance of intracranial meningioma in NF2

The PKD1 and PKD2 gene products are integral transmembrane proteins

Crystal structure of human short/branched chain acyl-CoA dehydrogenase (PDB file 2JIF). A homotetramer with bound butyryl-Co as substrate is represented.

A section of the cerebellum stained with Sevier-Munger preparation from the brain of a 10-year-old SCA1 patient with 82 CAG repeats who died after 6 years.

Effect of chenodeoxycholic acid feeding on the bilirubin levels of a 10-year-old boy with 3β-hydroxy-Δ5-oxidoreductase/isomerase deficiency. (From Setchell.

Aggregation-mediated passive sorting of secretory and membrane proteins during the biogenesis of a secretory granule in the TGN. (1) The luminal contents.

Urinary excretion (mg/h) of tryptophan derivatives following administration of oral L-tryptophan (about 70 mg per kilogram body weight) administered to.

A hypothetical model of the central apoptotic pathway

Free energy folding landscape for a hypothetical protein

Representation of Southern blot analysis revealing an expanding triplet repeat mutation in a myotonic dystrophy family. A grandmother and mother with the.

Progressive myopathy in a patient with type IIIa glycogen storage disease. The patient has a debrancher deficiency in both liver and muscle (subtype IIIa).

Model depicting the cycling of REP

Basal and peak serum thyrotropin (TSH) concentrations following intravenous administration of 500 μg thyrotropin-releasing factor (TRF) to subjects with.

Relation between the fractional catabolic rate (FCR) for plasma LDL and the number of LDL receptors on fibroblasts in patients with FH. The values for.

Model accounting for the mechanisms linking epipodophyllotoxin therapy, MLL fusion proteins, cell cycle progression, and the relaxation of cell cycle checkpoints,

Two children with LPI. The pictures were taken at the time of diagnosis. A, Child 12 years old. B, Child 6 years old. Note the prominent abdomen, hypotrophic.

Evolution of the ubiquitous α-, β-, and γ-crystallins and their structure. Details are described in the text. The α-crystallins are related to heat-shock.

Localization of WT1 mRNA expression in the developing kidney

Mechanism of NO synthesis

A comparison of normal cardiac anatomy (A) and the pathology of hypertrophic cardiomyopathy (B). Hypertrophic cardiomyopathy causes cardiomegaly (570 g;

Amino acid sequence and tentative structure for human prepro factor IX

Structure and mutation spectrum of BRCA1

Orofacial dyskinesia in a patient with Huntington's disease

Mechanism of NO synthesis

Photosensitive skin lesions on the face of a child with C4 deficiency

Expression of mutant human P5C dehydrogenase alleles in yeast

Karyotype of glioblastoma

The renin-angiotensin-aldosterone system

Hydrolysis of membrane-associated ceramide by acid ceramidase in the presence of saposins and bis(monoacylglycero)phosphate (BMP) in the acidic lysosomal.

Crystal structure of cdk2, cyclin A-cdk2, and cyclin A-cdk2-p27 complexes. A, The structure of monomeric cdk2. The T-loop is indicated in yellow, and the.

MRI contrast-enhancement and progression of X-ALD

Key structural elements of p53 DNA binding

Electrophoregram profiles showing the 35delG mutation of CX26 (GJB2) in an individual with recessively inherited hearing loss, and in his heterozygous.

Corneal opacity in a heterozygote observed by slit-lamp microscopy

Models of Fibrin Generation

FISH of glioblastoma. This interphase nucleus from a glioblastoma contains three chromosome 7 centromere signals (dark) and one centromere 10 signal (light).

A model of the protein C activation complex

Young female with OCA1B. The hair was white at birth

Overview of the TGF-β signaling pathway

Schematic representation of p53 molecule

The effects of apo E alleles on various lipoprotein parameters

Hepatic glucose production as a function of fasting plasma glucose levels. These studies involved the same individuals who were studied in Fig

Basic algorithm for cancer risk assessment that employs gene testing.

Schematic working model of the hepatic microsomal glucose-6-phosphatase system. ER = endoplasmic reticulum. This model is not meant to represent the actual.

Detection of XIST RNA in interphase cells from a normal male, a normal female, and a 49,XXXXX female by RNA fluorescence in situ hybridization. Male.

Structure and mutation spectrum of BRCA2

Composite megalosaccharide proposed for blood group substance

Complete chemical structures of the neutral glycosphingolipids that accumulate in Fabry disease. A, Globotriaosylceramide, the major accumulated substrate.

The PBDs can be caused by defects in peroxisomal matrix protein import or peroxisome membrane synthesis. Immunofluorescence microscopy shows that human.

DNA analysis on patients with STS deficiency

Presentation transcript:

More detailed view of the process shown in Fig. 178-1 More detailed view of the process shown in Fig. 178-1. Both plasminogen activators, tPA and uPA, are shown. Plasmin also activated tPA and uPA to form an amplification loop. PAI-1 binds to vitronectin, which stabilizes PAI-1 in the active form and localizes PAI-1 to the subendothelial matrix. Specific cellular receptors for uPA (uPAR), tPA, and plasminogen are indicated. Plasmin binds to fibrin, where it is protected from inhibition by α2AP. Fibrin also accelerates the activation of plasminogen by tPA. TAFI is activated by thrombin and inhibits fibrinolysis by removing lysine-binding sites for tPA and plasminogen. PAI-1 may form another link between hemostasis and fibrinolysis by inhibiting thrombin. Source: Disorders of the Fibrinolytic System, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: http://ommbid.mhmedical.com/DownloadImage.aspx?image=/data/books/971/ch178fg2.png&sec=62651085&BookID=971&ChapterSecID=62651077&imagename= Accessed: October 06, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved