Principles of Clinical Genetics Yogesh Ashok Sontakke
INDEX
Page numbers followed by f refer to figure and t refer to table
A
ABO blood group 1, 111
system 111
Achondroplasia 51, 53
Acid
alpha-glucosidase 61
elution test 78
Acyl-CoA dehydrogenase deficiency 61
Acyltransferase 56
Adenine 2931
Adenosine 32
deaminase 85
deficiency 87
Adenoviruses 83, 85, 86
Adrenal hyperplasia, congenital 61
Adult stem cells 89, 91, 91f, 92, 92t
Adult T-cell leukemia 72
Agarose gel electrophoresis 100
Albinism 52, 61
Alkaptonuria 1f, 52, 61
Allele 47, 111, 113
Alpha-1 anti-trypsin deficiency 87
Alport syndrome 54
Alzheimer's disease 87, 92
Ambiguous external genitals 27
Amenorrhea 24
Amino acid metabolism, disease of 61
Amniocentesis 25, 74, 75, 75f
Amniotic cavities 119
Amniotic fluid 10, 79, 89
index 79, 79t
Amplified fragment length polymorphism 40
Andersen disease 61
Anemia
aplastic 93
sideroblastic 58
Angelman syndrome 16
Aniridia 16, 108
Antibody 111, 111t, 114
Antigen 111, 111t, 114
Antioncogene 69
Aorta, coarctation of 24
Apoptosis 70
Arthritis
postgonococcal 116
rheumatoid 87, 116
Arylsulfatase B 62
Asthma 57
ATD angle 67, 67f
Athabascan brainstem dysgenesis syndrome 108
Autism 28
Autoimmune disorders 20
Autosomal dominant 49, 62
disorders 51t
inheritance 49, 51f, 53, 53t
Autosomal recessive 49, 62
disease 63
disorder 27, 52t
inheritance 52, 52f, 53, 53t
Azoospermia 25
B
Baldness 93
Barr body 7, 7f, 9, 10
number of 7, 7t
Barth syndrome 56
Becker's muscular dystrophy 56
Beta cells, development of 108
Biolistic particle delivery system 85
Blindness 56
Blood 10
group 111, 111t, 112
genetics 111
system 54
Bone marrow 10, 92
transplantation 93
Bort's test 77
Bosley-Salih-Alorainy syndrome 108
Bourneville-Pringle disease 51
Brachydactyly 108
Brain 92
Branched-chain-alpha-keto acid dehydrogenase 61
Breast cancer, hereditary 70
Bruton syndrome 56
Buccal smear 10, 25, 28
Burkitt's lymphoma 72
C
Cancer 69, 70t
breast 70
cervical 70
colon 70
colorectal 70
genetics 2, 69
genital 72
ovarian 70
type of 70
Carbamoyl phosphate synthetase 1 deficiency 61
Carbohydrate metabolism, disorders of 61
Carcinoma 108
hepatocellular 72
nasopharyngeal 72
thyroid 70
Cat eye syndrome 21
Cataract 56
Celiac disease 116
Cell
adhesion 70
cycle regulation 70
death 69
division 70, 107
growth, regulation of 71, 71f
proliferation 70
Centromere 4
transverse division of 19
Chargaff's rule 30, 33
Chorionic sacs 119
Chorionic villus sampling 10, 25, 74, 76, 76f
Chromosomal
aberration 1, 15, 20
classification of 15
disorders 48, 75
duplication 20
Chromosome 3, 8, 12
acrocentric 8
artificial 80
chemical composition of 7, 8
length of 12
morphology of 3
philadelphia 19
position of 12
ring 16, 20, 20f
shape of 12
structural classification of 8
structure of 4, 4f
submetacentric 8
telocentric 8
types of 9f
Cleft lip 57
Cleft palate 26, 57
Clinodactyly 23
Cloning, methods of 120
Codominant inheritance 57
Coloboma syndrome 108
Color blindness 56
Copper metabolism 62
disorders of 62
Cordocentesis 78
Cori's disease 61
Coronary artery disease 87
Cranial malformation 108
Cri-du-Chat syndrome 16, 17
Cubitus valgus 24
Cyclin-dependent kinase inhibitor 70
Cyclopia 27
Cystic fibrosis 52, 53, 82, 87
transmembrane regulator 52
Cytochrome B gene 58
Cytosine 2931
D
Deafness 93
Dendrimers 83, 86
Dental pulp 89, 92
Denver classification 12, 12t
Deoxynucleoside triphosphates 99
Deoxyribonucleic acid 1, 3, 8, 29, 58
Deoxyribose sugar 29
Depression 57
Dermal ridges, development of 64
Dermatoglyphics 64, 65, 68
applications of 64
Diabetes 93
mellitus 57
insulin independent 116
Dichorionic diamniotic twins 119
DiGeorge syndrome 16
Diploid 21
number 3
Distal interphalangeal creases 65
Distal palmar crease 65
Down syndrome 1, 2123, 27, 64, 67, 75, 77
Duchenne muscular dystrophy 56, 87
Dystrophin 56
gene, insertion of 87
Dystrophy, myotonic 51
E
Ectodermal dysplasia syndrome 108
Edward syndrome 21, 27
Embryoblast 89
Embryonic stem cell 89, 90, 90f
Endocytosis, receptor-mediated 83, 84
Enzyme 61, 104
role of 62
Epidermal growth factor 70, 71f
Epidermis 89
Epilepsy 57
Epithelia 92
Epstein-Barr virus 72
Erythroblastosis foetalis 112
Escherichia coli bacteria 104
Estriol 74, 77
Estrogen 25
Euchromatin 5, 6, 6f, 6t
Exocrine pancreas dysfunction 58
F
Facial hemangioma 6
Fanconi's anemia 87
Fatty acid oxidation, disorders of 61
Favism 61
Fetoscopy 78
Feulgen reaction 13
Flat feet 28
Fluorescent in situ hybridisation 10, 13, 40
Focal dermal hypoplasia 54
Foetal
anomalies 95
blood 10
sampling 74, 78
cells 74, 77
respiratory distress syndrome 79
Foetoscopy 74
Foetus 77, 78
position of 74
Folic acid 20
Follicle-stimulating hormone 25
Forebrain development, failure of 27
Fragile leukemic cells 10
Fragile X
mental retardation gene 54
syndrome 28, 54
G
Galactose-1-phosphate uridyl transferase 62
Galactosemia 58
classic 62
Galton classification 66
Gaucher's disease 62, 87
Gene 34
bank 41
cluster of 115
complete set of 48
delivery 83
expression 37
gun 83, 85
jumping 1, 39
mapping 39
uses of 40
multiple 111
polydactyly 59
therapy 80, 81, 87
disadvantages of 87
milestones of 81t
principles of 80
transfer 83, 85, 86
chemical methods of 86
methods of 83t
techniques 82
Genetic
counseling 95, 96, 96f
steps of 96, 97
diseases 93
disorder 48
mapping 40
marker 40
Genitalia 27
Genotype 35, 43, 48, 112
Germ cell 82
mutation 38, 39
Germline gene therapy 82, 82t
Gestational age, determination of 74
Giemsa stain 11
Glacial acetic acid 11
Glaucoma 57
Glioblastoma 70
Glucocerebrosidase 62
Glucose 6-phosphate dehydrogenase 61
deficiency 56
Glutaric aciduria 61
Glutaryl-CoA dehydrogenase 61
Glycogen
branching enzyme 61
debranching enzyme 61
storage disease 1, 61
Goltz syndrome 54
Gonadal dysgenesis 24, 109
Grafts, types of 114
Guanine 2931
Guanosine triphosphate 32
Guthrie test 63
Guttmacher syndrome 108
Gynecomastia 25
H
Haploid 21
number 3
Hard-foot-genital syndrome 108
Heart
defects, congenital 57
sound 74
Hemochromatosis 52
Hemoglobin beta gene 52
Hemophilia
A 56
B 56, 87
Hepatitis
autoimmune 116
B virus 72
C virus 72
Hermaphroditism 27
Herpes simplex virus 83, 85, 86
Hers disease 61
Heterochromatin 5, 6, 6f, 6t, 13
types of 5
Hexosaminidase A deficiency 62
High-resolution banding technique 13
Hodgkin's lymphoma 72
Homeobox genes 107
Homocystinuria 61
Homogentisate 1, 2-dioxygenase gene 52
Homogentisic acid oxidase 61
Homoploid 21
Horseshoe kidney 25
Human
chorionic gonadotropin 77, 78
genetics 1t
genome project 40
compilation of 1
HOX gene disorders 108t
leukocyte antigen 116t
complex 116
system 115
papilloma virus 72
T-lymphotropic virus 72
Hunter syndrome 62
Huntington chorea 51
Hurler syndrome 62
Hydatidiform mole 110
complete 110
partial 110
Hydroxylation 37
Hyperammonemia, cerebroatrophic 54
Hypercholesterolemia 51
familial 87
Hyperplasia, adrenal 27
Hypertension 57
Hyperuricemia 56
Hypogonadism 25
Hypomelia 6
Hypotonia 23
Hypotrichosis 6
Hypoxanthine 29
guanine phosphoribosyl transferase 56, 61
I
Iduronate sulfatase 62
Immunity 114
cellular 114
humoral 114
In vitro fertilisation 26, 79, 97
Indian National Gene Bank 41
Infertility 24, 25
Insemination, artificial 97
Intestine 89
Iris, heterochromia of 108
Isochromosome 16, 19, 20f
J
Juvenile gout 56, 61
K
Kaposi's sarcoma 72
Karyotype 7, 21, 26, 24
Kearns-Sayre syndrome 58, 61
Kettering test 77
Kidney 108
Kinetochore 4
Kleihauer-Betke test 78
Klinefelter syndrome 7, 21, 25, 26f, 67
L
Langer-Giedion syndrome 16
Leber's hereditary optic neuropathy 58
Lecithin-sphingomyelin ratio 79t
Lejeune's syndrome 17
Lesch-Nyhan syndrome 56, 61
Leukocoria 56
Li-Fraumeni syndrome 72, 72f
Limb deformities 75
Lipoprotein, low-density 51, 60, 87
Liposome 83
mediated gene transfer 83, 83f
Liver 89
cirrhosis of 93
glycogen phosphorylase 61
Lymphedema over limbs 24
Lymphocyte syndrome 116
Lymphoid cells 89
Lyon hypothesis 7
Lysosomal
enzyme acid sphingomyelinase 62
storage disorder 52
M
Macroglossia 22
Major histocompatibility complex 115
Mandibulofacial dysostosis 51
Maple syrup urine disease 61
Marfan syndrome 51, 53
Marie-tooth disease 108
Maroteaux-Lamy syndrome 62
Maternal
blood screening test 77
cell contamination 77
serum alpha-foetoprotein 77, 78
McArdle disease 61
Melanoma 70
malignant 70
Mendel's laws 43, 46
biological significance of 46
Mendelian disorders 48
Menkes disease 62
Mental
disorders 63
retardation 26, 28
Messenger ribonucleic acid 114, 120
Metacentric chromosome 8
Methylation 7
Micrognathia 22
Microphthalmia 27
Miller-Dieker syndrome 16
Mitochondrial
functions, disorders of 61
inheritance 58, 58f
myopathy 58
Monosomy 21
Mosaicism 25
Motor neuron 1 gene, survival of 52
Mucopolysaccharides 62
Multigenic disorders 87
Multiple endocrine neoplasia 70
Muscle
glycogen phosphorylase 61
phosphofructokinase 61
Muscular dystrophy 93
Mutation 38, 108
causes of 38
chromosomal 39
classification of 39t
Myeloid leukemia, chronic 19
Myocardial infarction 93
Myogenesis 108
N
Neonatal heel prick 63
Neural tube defects 57
Neurofibromatosis 51, 70
Neutral endopeptidase, phosphate-regulating 54
Neutrophil 7
Niemann-Pick disease 62
Norrie disease 56
Nuchal translucency 74
Nucleoside 29
Nucleotide excision repair 52
Nutritional deficiencies 20
Nystagmus, sensory 26
O
Okazaki fragments 41
Oligodontia 108
Oligonucleotides 83, 86
Oncogenes 69, 70t
Oncovirus 72, 72t
Optic nerve 108
Organic acid metabolism, disorders of 61
Oropharyngeal squamous cell carcinoma 70
Osteogenesis imperfecta 51
Osteoporosis 25
Osteosarcoma 70
Ovarian follicles 24
Ovary 78
Ovotesticular disorder 27
Ovum 3, 82
P
Pallister-Killian syndrome 21
Palmar creases 65, 66f
Parkinson's disease 92, 109
Patau syndrome 21, 26
Patchy white skin pigmentation 108
Pearson syndrome 58
Pedigree
charting, advantages of 48
symbols 49f, 50f
Pentose sugar 29
Percutaneous umbilical blood sampling 78
Peroxisomal function, disorders of 62
Peter's anomaly 27, 108
Phenotype 43, 48
Phenylalanine hydroxylase 52, 61, 62
deficiency 1, 63
Phenylketonuria 1, 52, 53, 58, 61, 62
Phosphate molecule 29
Phytohemagglutinin 10
Placenta 77, 78, 89, 92
localisation of 74
Plasma protein A, pregnancy-associated 74, 77
Pleiotropy 58
Polycystic kidney disease 51
Polydactyly 26
Polygenic inheritance 1, 56
Polymerase chain reaction 99, 99f, 101f
applications of 100
Polyplexes 83, 86
Polyploid 21
Polyposis, familial adenomatous 70
Polysyndactyly syndrome 108
Pompe's disease 61
Porphobilinogen deaminase 61
Porphyria, acute intermittent 61
Prader-Willi syndrome 16
Pregnancy
confirmation of 74, 79
molar 110
Primers set 99
Progenitor cells 89, 89t
Progesterone 25
Protein synthesis 1
Pseudogenes 39
Pseudohermaphroditism 27
Pseudouracil 31
Puberty, delayed 25
Pyloric stenosis 57
Q
Quad screening test 77, 78t
R
Radioulnar synostosis 108
Real-time polymerase chain reaction 101
Red blood cells 111
Retina 89
Retinal ganglionic cell degeneration 58
Retinoblastoma 70
Retrotransposed pseudogenes 39
Retroviruses 83, 85, 86
Rett syndrome 54f
Reverse transcription polymerase chain reaction 102f
Ribonucleic acid 1, 31
Robert syndrome 6
Rocker bottom feet 26
Rubinstein-Taybi syndrome 16
S
Schiff's reagent 13
Schizophrenia 57, 109
Sclerosis, tuberous 51
Scoliosis 25
Severe combined immunodeficiency syndrome 85, 93
Sex chromatin, study of 10
Sickle cell
anemia 52
trait 47
Signal transduction genes 70
Simian crease 23, 23f, 26, 65
Single gene
disorders 48
inheritance 49
Single nucleotide polymorphism 40
Sjögren syndrome 116
Skeletal muscle 89
Sly syndrome 62
Smith-Magenis syndrome 16
Somatic cell 82
mutations 38
Somatic gene therapy 82
Sonoporation gene therapy 85
Sperm 3, 82
Spina bifida 75
Spinal cord 92, 112
injury 93
Spinal muscular dystrophy 52
Spondylitis 57
ankylosing 116
SRY gene 109
mutation of 109
role of 109, 109f
Stem cell 88, 89, 89t, 92, 93f
classification of 89
plasticity of 92
pluripotent 92
properties of 88, 88f
sources of 89
therapy 88
applications of 92
types of 89
Steroid
metabolism, disorders of 61
sulfatase enzyme gene 56
Strabismus 24
Stroke 92
Suwon crease 65
Syndactyly 108
Systemic lupus erythematosus 116
T
Tafazzin gene 56t
Tandem mass spectrometry 63
Tarui's disease 61
Taurodontic teeth 25
Tay-Sachs disease 52, 62
Teratoma cells 89
Testicular biopsy 25
Testosterone 25
low level of 25
therapy 26
Tetrasomy 21
Thalassemia 93
Thenar crease 65
Thrombocytopenia 108
Thymine 29, 30
Thyroid gland development 108
Traumatic brain injury 92
Treacher Collins syndrome 51
Triple screening test 77, 77t
Triple X syndrome 21
Trisomy 21
13 21
18 21
21 21
8 21
X 21
Trophoblast cells, transcervical retrieval of 74
Trypsin 12
Tumors 87
suppressor gene 69, 70t, 87
functions of 69
Turner syndrome 7, 9, 19, 21, 23, 24f, 67, 75
Twins 117
conjoined 119
dizygotic 117, 118, 118t
monoamniotic 119
monochorionic 119
monozygotic 117, 118, 118t, 119, 119t, 120
parasitic 119
types of 117, 118f
Tyrosine 63
deficiency 63
kinase 56
U
Ullrich-Turner syndrome 23
Umbilical
cord 89, 92
vein sampling 78
Urea cycle, disorders of 61
Urine strip test 79
Uveitis, acute anterior 116
V
Ventricular septal defects 24
Viral vectors, disadvantages of 85
Viruses, adeno-associated 83, 85, 86
Vitamin D resistant rickets 54
von Gierke's disease 61
W
Waardenburg syndrome 51, 108
Warkany syndrome 21
Warts 72
Watson and Crick model 30
William syndrome 16
Wilms’ tumor 6, 87
Wilson disease 62
Wolf-Hirschhorn syndrome 16
Wound healing 93
Wrist creases 65
X
Xanthine 29
Xeroderma pigmentosa 41, 52
X-linked
agammaglobulinemia 56
dominant 49
inheritance 53, 54, 54t
hypophosphatemia 54
ichthyosis 56
recessive 49, 62
inheritance 54, 55f, 56t
XXX syndrome 7, 27
Y
Y chromosome, role of 1
Y-linked
disorders 49
inheritance 55
Z
Zinc finger genes 108
Zygote 117
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