Space-filling model of the 24-meric cubic core and its constituent trimers. (Left panel) The trimer, where the three catalytic (inner-core) domains (depicted.

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
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.
Physical map of a human chromosome
Patterns of genetic change in neuroblastomas
Light micrographs of a normal human retina (left), a retina with retinitis pigmentosa (center), and a retina with end-stage retinitis pigmentosa (right).
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
The bottom panel depicts the "breakdown" of the barriers in a cancer cell such that the transcriptionally repressive chromatin and DNA methylation all.
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
Serum and urine uric acid
Distribution of CpG dinucleotide in the human genome and differences in methylation patterns between normal and tumor cells. In the majority of the mammalian.
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.
Urinary excretion (mg/h) of tryptophan derivatives following administration of oral L-tryptophan (about 70 mg per kilogram body weight) administered to.
Neuropathology. Photomicrographs of the cerebellum
Free energy folding landscape for a hypothetical protein
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.
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.
Depiction of meiotic crossing over and linkage analysis
Anteroposterior radiographs of right tibia and fibula of case 2 at 2 years of age and left tibia and fibula of case 3 at age 6 years. Features of osteopetrosis.
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
High-pressure liquid chromatography of deproteinized cerebrospinal fluid (CSF), plasma, and urine of an ADSL-deficient patient (left panels) and of a control.
Recovery of E1 activity in E1 variants carrying type IA MSUD mutations as a function of increasing TMAO concentrations. Y368C-α (▪), F364C-α (•), and Y393N-α.
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.
Metabolism of mevalonic acid and early steps in the biosynthesis of cholesterol. The structures and names of the intermediates in the synthetic pathway.
DNA analysis on patients with STS deficiency
Presentation transcript:

Space-filling model of the 24-meric cubic core and its constituent trimers. (Left panel) The trimer, where the three catalytic (inner-core) domains (depicted in red, yellow, and blue) from different E2 subunits form the basal functional and assembly unit. N and C = the amino and carboxy termini, respectively. (Right panel) The 24-meric cubic core, where eight trimers are assembled into a hollow cube (~130-Å edge), with each trimer occupying one of the eight vertices. The ovals (in white) depict trimer-trimer interfaces comprising the C-termini from the neighboring trimers. Substrate CoA enters the 30-Å-long active-site channel from inside the cube (green arrow), whereas the second substrate LBD approaches the active-site channel from the outside. Source: Maple Syrup Urine Disease (Branched-Chain Ketoaciduria), 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: https://ommbid.mhmedical.com/DownloadImage.aspx?image=/data/books/971/ch87fg1.png&sec=62675443&BookID=971&ChapterSecID=62675436&imagename= Accessed: October 05, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved

Feedback: Privacy Policy Feedback
Do Not Sell My Personal Information
About project: SlidePlayer Terms of Service

© 2024 SlidePlayer.com. Inc. All rights reserved.