Table of Contents

0. Executive Summary 23

1. Introduction 25

Definitions and scope of the subject 25

Historical evolution of molecular diagnostics 25

Molecular biology relevant to molecular diagnostics 26

DNA 26

DNA polymerases 26

Restriction endonucleases 27

DNA methylation 27

RNA 28

RNA polymerases 28

Non-coding RNAs 28

DNA transcription 29

Chromosomes 29

Telomeres 30

Mitochondrial DNA 30

Genes 31

The genetic code 31

Gene expression 31

The human genome 32

Variations in the human genome 32

Variations in DNA sequences 32

Single nucleotide polymorphisms 33

Haplotyping 33

Copy number variations in the human genome 34

Genotype and haplotypes 35

Insertions and deletions in the human genome 35

Complex chromosomal rearrangements 36

Large scale variation in human genome 36

Structural variations in the human genome 37

Replication of the DNA helix 37

Transposons 38

Proteins 38

Proteomics 38

Monoclonal antibodies 39

Aptamers 39

Basics of molecular diagnostics 39

Tracking DNA: the Southern blot 39

Pulsed-field gel electrophoresis 40

DNA Probes 40

The polymerase chain reaction 41

Basic Principles of PCR 41

Target selection 41

Detection of amplified DNA 41

Impact of human genome project on molecular diagnostics 42

Mapping and sequencing of structural variation from human genomes 42

1000 Genomes Project 43

Human Variome Project 44

Role of bioinformatics in molecular diagnostics 44

Systems biology approach to molecular diagnostics 45

Biomarkers 45

Applications of molecular diagnostics 45

2. Molecular Diagnostic Technologies 49

Introduction 49

DNA sample collection and extraction 49

Blood samples 49

Buccal swabs and saliva 49

Urine samples for transrenal DNA 50

Manual vs automated DNA extraction 50

Sample preparation 51

Pressure Cycling Technology 51

Membrane immobilization of nucleic acids 51

Automation of sample preparation in molecular diagnostics 52

ABI PRISM 6700 Automated Nucleic Acid Workstation 52

BioRobot technology 52

COBAS AmpliPrep System 52

GENESIS FE500 Workcell 52

GeneMole 53

PCR BioCube 53

QIAsymphony 53

Tigris instrument system 53

Techniques for sample preparation that are suitable for automation 54

Xtra Amp Genomic DNA Extraction 54

Extraction of DNA from paraffin sections 54

Dynabead technology 54

Pressure Cycling Technology 54

SamPrep 55

Use of magnetic particles for automation in genome analysis 55

Companies involved in nucleic acid isolation 56

Novel PCR methods 57

Addressing limitations of PCR 57

CAST-PCR 57

Combined PCR-ELISA 57

Convection PCR 58

Digital PCR 58

Emulsion PCR 59

ExCyto PCR 59

Long and accurate PCR 60

Multiplex PCR 60

Real-time PCR systems 60

Dyes used in real-time PCR 61

Commercially available real-time PCR systems 61

LightCycler PCR system 62

LightUp probes based on real-time PCR 62

READ™ real-time PCR method 63

StellARray™ technology 63

7500 Fast Dx Real-Time PCR Instrument 63

Applications of real-time PCR 63

Limitations of real-time PCR 64

Quantitative PCR for accurate low level DNA analysis 64

Guidelines for real-time qPCR 65

Future applications of real-time qPCR 65

Reverse transcriptase (RT)-PCR 66

Standardized reverse transcriptase PCR 66

Single cell PCR 67

LATE-PCR 67

COLD-PCR 67

AmpliGrid-System 68

Post-PCR genotyping methods 68

High-resolution melt analysis 69

Monitoring of gene amplification in molecular diagnostics 69

Non-PCR nucleic acid amplification methods 69

DNA probes with conjugated minor groove binder 69

Dynamic Flux Amplification 70

Isothermal reaction for amplification of oligonucleotides 70

ICAN (Isothermal and Chimeric primer-initiated Amplification of Nucleic Acids) 70

Linked Linear Amplification 71

Multiplex Ligation-Dependent Probe Amplification 71

Rapid analysis of gene expression 71

Rolling circle amplification technology 72

Gene-based diagnostics through RCAT 73

RCAT-immunodiagnostics 73

RCAT-pharmacogenomics 74

Circle-to-circle amplification 74

Ramification amplification method 74

Single Primer Isothermal Amplification 75

Transcription mediated amplification 75

WAVE nucleic acid fragment analysis system 75

Technologies for signal amplification 76

3 DNA dendrimer signal amplification 76

Hybridization signal amplification method 77

Signal mediated amplification of RNA technology 78

Invader assays 79

Hybrid Capture technology 80

Branched DNA test 81

Tyramide signal amplification 81

Non-enzymatic signal amplification technologies 81

Direct molecular analysis without amplification 82

Trilogy™ Platform 82

Direct detection of dsDNA 83

Multiplex assays 84

Fluorescent in situ hybridization 84

FISH technique 85

Applications of FISH 85

Modifications of FISH 86

Direct visual in situ hybridization 86

Direct labeled Satellite FISH probes 86

Comparative genomic hybridization 87

Primed in situ labeling 87

Interphase FISH 87

FISH with telomere-specific probes 88

Multicolor FISH 88

Simultaneous Ultrasensitive Subpopulation staining/Hybridization In situ 88

Automation of FISH 89

Companies involved in FISH diagnostics 89

RNA diagnostics 90

Branched-chain DNA assay for measurement of RNA 91

Cycling probe technology 91

Invader RNA assays 91

Linear RNA amplification 92

Non-isotopic RNase cleavage assay 92

Nucleic acid sequence-based amplification 92

Q Beta replicase system 94

RNAScope 94

Solid Phase Transcription Chain Reaction 95

Transcriptome analysis 95

Visualization of mRNA expression in vivo 95

MicroRNA diagnostics 96

Real-time PCR for expression profiling of miRNAs 96

Microarray vs quantitative PCR for measuring miRNAs 96

Use of LNA to explore miRNA 97

Nuclease Protection Assay to measure miRNA expression 97

Microarrays for analysis of miRNA gene expression 97

Modification of in situ hybridization for detection of miRNAs 98

Whole genome amplification 99

Companies that provide technologies for whole genome amplification 99

QIAGEN’s Repli-G system 99

GenomePlexÔ Whole Genome Amplification 100

DNA sequencing 100

Companies involved in sequencing 101

Applications of next generation sequencing in molecular diagnostics 103

Companies developing sequencing for molecular diagnostics 103

Genome-wide approach for chromatin mapping 104

Mitochondrial sequencing 105

Identification of unknown DNA sequences 105

Optical mapping 105

Gene expression analysis 106

Gene expression profiling on whole blood samples 107

Gene expression patterns of white blood cells 107

Gene expression profiling based on alternative RNA splicing 107

MAUI (MicroArray User Interface) hybridization 108

Monitoring in vivo gene expression by molecular imaging 108

Serial analysis of gene expression (SAGE) 108

Single-cell gene expression analysis 109

T cell receptor expression analysis 110

Tangerine™ expression profiling 110

Whole genome expression array 110

Ziplex™ system 111

Companies involved in gene expression analysis 111

Peptide nucleic acid technology 112

Use of PNA with fluorescence in situ hybridization 113

PNA and PCR 113

Use of PNA with biosensors 114

PNA-based PD-loop technology 114

PNA-DNA hybrid quadruplexes 115

Companies involved in PNA diagnostics 115

Locked nucleic acids 115

Zip Nucleic Acids 116

Electrochemical detection of DNA 117

Mediated nucleic acid oxidation 117

Detection of hybridized nucleic acid with cyclic voltametry 118

Electrochemical detection based on Toshiba’s CMOS technology 118

Concluding remarks on electrochemical DNA detection 118

Bead-based assay platforms 118

Scorpions™ technology 119

The Scorpions reaction 119

Applications of Scorpions 120

Nucleic acid lateral flow molecular diagnostics 120

3. Biochips, Biosensors, and Molecular Labels 123

Introduction to biochip technology 123

Applications of biochips in diagnostics 123

GeneChip 124

GeneChip Human Genome Arrays 125

AmpliChip CYP450 125

Electronic detection of nucleic acids on microarrays 125

Microchip capillary electrophoresis 126

Strand displacement amplification on a biochip 126

Rolling circle amplification on microarrays 126

LiquiChip-RCAT 126

Fast PCR biochip 127

Multiplex microarray-enhanced PCR for DNA analysis 127

Multiplexed Molecular Profiling 127

Universal DNA microarray combining PCR and ligase detection reaction 128

Genomewide association scans 128

Whole genome chips/microarrays 129

Transposon insertion site profiling chip 129

Standardizing the microarrays 130

Companies involved in developing biochip technology for diagnostics 130

Future of biochip technology for molecular diagnostics 131

Microfluidic chips 132

Fish-on-chip 132

Lab-on-a-chip 132

LabCD 133

Micronics' microfluidic technology 133

Microfluidic chips/arrays using PCR 133

Microfluidic automated DNA analysis using PCR 133

Digital PCR Array 133

Digital PCR on a SlipChip 134

Microfluidic chips integrated with RCAT 134

Microfluidic chips integrated with PET 134

Companies developing microfluidic technologies 135

Biosensor technologies 135

Classification of biosensor technologies 136

DNA-based biosensors 137

DNA hybridization biosensor chips 137

PCR-free DNA biosensor 137

DNA based biosensor to detects metallic ions 138

Genetically engineered B lymphocytes 138

Biosensors immunoassays 138

PNA (peptide nucleic acid)-based biosensors 139

Protein-based biosensors 139

Antibody biosensors 139

Cell-based biosensors (cytosensors) 139

Multicell biosensors 140

Microbial biosensors 140

Optical biosensors 141

Surface plasmon resonance technology 141

Label-free optical biosensor 142

Microsensors using with nano/microelectronic communications technology 142

Electrochemical sensors 142

Enzyme electrodes for biosensing 142

Conductometric sensors 143

Electrochemical genosensors 143

Electrochemical nanobiosensor 143

Bioelectronic sensors 144

Phototransistor biochip biosensor 144

Ribozyme-based sensors 144

RiboReporters 145

Concluding remarks and future prospects of biosensor technology 145

Companies developing biosensors for molecular diagnostics 146

Molecular labels and detection 147

Detection technologies for molecular labels 148

Fluorescence and chemiluminescence 148

Fluorescence technologies for label detection 149

Companies with fluorescence and chemiluminescence products 149

Molecular beacons 150

The Green fluorescent protein 151

Multiophoton detection radioimmunoassay 152

Multi-pixel photon counter 152

Enzyme labels and detection by fluorescence 152

Phase-sensitive flow cytometry 153

Microtransponder-based DNA diagnostics 153

Laboratory Multiple Analyte Profile 154

Multiple labels 154

Protein-DNA chimeras for detection of small numbers of molecules 155

Single molecule detection 155

Atomic force microscopy 156

Capillary electrophoresis 156

Confocal laser scanning 156

nCounter Analysis System 156

Spectrally resolved fluorescence lifetime imaging microscopy 157

Molecular imaging 157

Basic research in molecular imaging 158

Devices for molecular imaging 158

Molecular imaging in clinical practice 158

Challenges and future prospects of molecular imaging 159

Companies involved in molecular imaging 159

Nanobiotechnology for molecular diagnostics 159

Magnetic nanoparticles 160

Gold nanoparticles 161

Quantum dot technology 162

Nanotechnology on a chip 163

Nanogen's NanoChip 163

Fullerene photodetectors for chemiluminescence detection on microfluidic chip 163

Diagnostics based on nanopore technology 164

Nanosensors 164

Detection of cocaine molecules by nanoparticle-labeled aptasensors 164

Nanosensors for glucose monitoring 165

PEBBLE nanosensors 165

Quartz nanobalance biosensor 165

Cantilever arrays 165

Resonance Light Scattering technology 166

DNA nanomachines for molecular diagnostics 167

Nanobarcodes technology for molecular diagnostics 167

Qdot nanobarcode for multiplexed gene expression profiling 167

Role of nanobiotechnology in improving molecular diagnostics 168

Companies involved in nanomolecular diagnostics 168

Concluding remarks about nanodiagnostics 171

Future prospects of nanodiagnostics 171

4. Proteomic Technologies for Molecular Diagnostics 173

Introduction 173

Proteomic technologies 173

Biomarkers of disease 173

Proteomic tools for biomarkers 173

Search for biomarkers in body fluids 174

Captamers with proximity extension assay for proteins 174

Cyclical amplification of proteins 174

Detection of misfolded proteins by ELISA with exponential signal amplification 175

Diagnostics based on designed repeat proteins 175

Differential Peptide Display 175

Light-switching excimer probes 176

MALDI-TOF MS 176

Molecular beacon aptamer 177

Molecular beacon assay 177

Proteomic patterns 178

Real-time PCR for protein quantification 178

Protein biochip technologies 179

ProteinChip 180

LabChip for protein analysis 180

TRINECTIN proteome chip 181

Protein chips for antigen-antibody interactions molecular diagnostics 181

Microfluidic devices for proteomics-based diagnostics 181

Nanotechnology-based protein biochips/microarrays 182

Nanoparticle protein chip 182

Protein nanobiochip 182

Protein biochips based on fluorescence planar wave guide technology 182

New developments in protein chips/microarrays 183

Antibody microarrays 183

Aptamer-based protein biochip 184

Multiplexed Protein Profiling on Microarrays 184

Proteomic pattern analysis 184

Single molecule array 185

Viral protein chip 185

Commercial development of protein chips for molecular diagnostics 185

Proteome Identification Kit 187

Laser capture microdissection (LCM) 187

LCM technology 187

Applications of LCM in molecular diagnostics 188

Proteomic diagnosis of CNS disorders 188

Cerebrospinal fluids tests based on proteomics 188

Urine tests for CNS disorders based on proteins in urine 189

Diagnosis of CNS disorders by examination of proteins in the blood 189

Diagnosis of CNS disorders by examination of proteins in tears 190

Role of proteomics in the diagnosis of Alzheimer's disease 190

Role of proteomics in the diagnosis of Creutzfeldt-Jakob disease 191

Future prospects of use of proteomics for diagnosis of CNS disorders 191

Concluding remarks on the use of proteomics in diagnostics 191

5. Molecular Diagnosis of Genetic Disorders 193

Introduction 193

Cytogenetics 194

FISH with probes to the telomeres 194

Single copy FISH probes 194

Comparative genomic hybridization 195

Use of biochips in genetic disorders 195

Representational oligonucleotide microarray analysis 196

SignatureChip®-based diagnostics for cytogenetic abnormalities 196

Diagnosis of genomic rearrangements by multiplex PCR 196

Quantitative fluorescent PCR 196

Mutation detection technologies 197

PCR-based methods for mutation detection 198

Cleavase Fragment Length Polymorphism 198

Direct dideoxy DNA sequencing 198

Digital Genetic Analysis (DGA) 198

Fluorescence-based directed termination PCR 199

Heteroduplex analysis 199

Restriction fragment length polymorphism 200

Single-stranded conformation polymorphism (SSCP) analysis 200

TaqMan real-time PCR 201

Non-PCR methods for mutation detection 201

Arrayed primer extension 201

BEAMing (beads, emulsion, amplification, and magnetics) 201

ELISA-protein truncation test 202

Enzymatic mutation detection 202

Specific anchor nucleotide incorporation 202

Conversion analysis for mutation detection 203

Biochip technologies for mutation detection 203

Combination of FISH and gene chips 204

Haplotype Specific Extraction 204

Technologies for SNP analysis 204

DNA sequencing 205

Electrochemical DNA probes 206

Use of NanoChip for detection of SNPs 206

Single base extension-tag array 206

Laboratory Multiple Analyte Profile 207

SNP genotyping with gold nanoparticle probes 207

PCR-CTPP (confronting two-pair primers) 207

Peptide nucleic acid probes for SNP detection 208

SNP genotyping on a genome-wide amplified DOP-PCR template 208

Pyrosequencing 208

Reversed enzyme activity DNA interrogation test 209

Smart amplification process version 2 209

Zinc finger proteins 210

UCAN method (Takara Biomedical) 210

Biochip and microarray-based detection of SNPs 210

SNP genotyping by MassARRAY 210

Electronic dot blot assay 211

Biochip combining BeadArray and ZipCode technologies 211

SNP-IT primer-extension technology 211

OmniScan SNP genotyping 212

Affymetrix SNP genotyping array 212

Concluding remarks on SNP genotyping 212

Limitations of SNP in genetic testing 213

Haplotyping versus SNP genotyping 213

Nanofluidics technology for high throughput SNP genotyping 213

Companies involved in developing technologies/products for SNP analysis 214

Role of copy number variations in genetic diagnostic testing 215

CNVs in various diseases 215

CNVs in genetic epilepsy syndromes 215

CNVs associated with schizophrenia 216

Methods for determination of CNVs 216

Digital array for CNV detection 216

Wellcome Trust Case Control Consortium CNV typing array 217

CNVer algorithm for CNV detection 217

Study of rare variants in pinpointing disease-causing genes 217

Prenatal DNA diagnosis 218

Amniocentesis 218

Chorionic villus sampling 218

Separating fetal cells in maternal blood for genetic diagnosis 218

Antenatal screening for Down's syndrome 219

Fetal DNA in maternal blood 219