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. Author manuscript; available in PMC: 2014 Jul 15.
Published in final edited form as: Nat Rev Genet. 2012 Dec;13(12):853–866. doi: 10.1038/nrg3336

Table 1.

Partial list of genes and genetic elements showing HLS changes

Gene/Element Name Mechanism of Change Proposed Phenotype Relative Phenotypic Certainty Possible Gene-Associated Disease Refs
Androgen receptor (AR) Deletion of regulatory DNA/Expression Change Loss of sensory vibrissae and penile spines Likely Androgen insensitivity, hypospadias 1, spinal and bulbar muscular atrophy of Kennedy, prostate cancer susceptibility 8
Apolipoprotein C-I (APOC1) Pseudogene Unknown N/A Alzheimer’s severity, atherosclerosis and coronary heart disease 5053
Aquaporin 7 (AQP7) Copy number increase Energy (glycerol) storage/transport/utilizat ion; increased sweating Plausible Nonfunctional glycerol response to exercise 2, 3, 18, 40, 5456
Asp (abnormal spindle) homolog, microcephaly associated (ASPM) Positive selection Increased Brain Size Plausible Microcephaly 57, 58
CDK5 regulatory subunit associated protein 2 (CDK5RAP2) Positive selection Increased Brain Size Plausible Microcephaly 58, 59
Chemokine (C-C motif) ligand 3-like 1 (CCL3L1) Novel gene variant Immune function (chemokine for lymphocytes and macrophages) Likely HIV/AIDs susceptibility, Kawasaki Disease, Rheumatoid Arthritis, susceptibility to Chronic hepatitis C infection 56
Cholinergic receptor, muscarinic 3 (CHRM3) Alternative splicing/Expression change/Novel exon Change in human reproduction Plausible Eagle-Barrett Syndrome 60
Cholinergic receptor, nicotinic alpha 7 and FAM7A fusion (CHRFAM7A) Copy number increase Higher brain function Plausible P50 sensory gating deficit 18, 40, 56, 61
Creatine kinase brain (CKB) Expression change Metabolic changes in brain Plausible Multiple sclerosis 4, 62
Cytidine Monophosphate Acetylneuraminic acid hydroxylase (CMAH) Pseudogene Changed sialic acid composition on all cells in the body/Secondary effects in multiple systems Definite Duchenne muscular dystrophy, Red-meat related carcinoma risk. 63, 64
Cytochrome c oxidase subunit Va (COX5A) Amino acid change/Positive Selection Mitochondrial metabolism Plausible 65
Dopamine receptor D5 (DRD5) Copy number increase Regulation of mood, memory, learning, attention, movement Likely DRD5 deficiency, ADHD, primary cervical dystonia 18, 56
DUF1220/Neuroblastoma breakpoint factor (NBPF) family Protein domain copy number increase (hyperamplification) Brain size Likely Microcephaly, macrocephaly 18, 23, 24, 40, 66. 67
Fc fragment of IgG, high affinity 1a, receptor (FCGR1A) Copy number increase Immune function Plausible IgG receptor I phagocyte deficiency 40, 56
Follicle stimulating hormone receptor (FSHR) Positive selection Decreased gestation/Birth timing Plausible Amenorrhea, infertility, ovarian dysgenesis type 1 (premature ovarian failure), ovarian hyperstimulation syndrome 68, 69
Forkhead box P2 (FOXP2) Amino acid change/Positive Selection Language/speech development and increased length of dendrite spines Definite Speech-language disorder-1 70
Forkhead box D4 (FOXD4) Novel Gene Variant Nervous system development Plausible Dilated cardiomyopathy, suicidality, OCD 56
General transcription factor 2-I repeat domain-containing protein 2 (GTF2IRD2) Copy number increase Unknown N/A Williams-Beuren syndrome 18, 56
Growth arrest and DNA-damage-inducible, gamma (GADD45G) Deletion of regulatory DNA/Expression change Expansion of human forebrain Plausible Thyroid carcinoma 8
Glutamate receptor, ionotropic, N-methyl-D-aspartate 3A (GRIN3A) Amino acid change/Positive Selection Learning and memory Plausible Unknown 71
Glutamate receptor, ionotropic, N-methyl-D-aspartate 3B (GRIN3B) Amino acid change/Positive Selection Higher brain function Plausible Unknown 71
Human accelerated conserved noncoding region 1 (HACNS1) Positive Selection Changes in development of anterior wrist and thumb Likely Unknown 9
Human accelerated region 1 forward (HAR1f) Positive Selection Development of neocortex Plausible Unknown 14
Mannose receptor C, type 1 (MRC1) Novel gene variant Recovery from inflammation, regulator of glycoprotein homeostasis Plausible Leprosy manifestation 56
Microcephalin 1 (MCPH1) Positive Selection Brain Size Plausible Microcephaly 58, 72
Myosin heavy chain 16 (MYH16) Pseudogene Cranio-facial musculature and morphology Plausible Unknown 73
Neutrophil cytosolic factor I (NCFI) Copy number increase Phagocyte generation of superoxides Likely Chronic granulomatous disease, Williams-Beuren syndrome 56
NLR family, apoptosis inhibitory protein (NAIP) Copy number increase Inhibition of apoptosis Likely Spinal muscular atrophy 18, 40, 56
Occludin (OCLN) Copy number increase Regulation of TGF-beta, cell migration Likely Susceptibility to hepatitis C, Band-like calcification with simplified gyration and polymicrogyria 18, 56, 74
p21 protein (Cdc42/Rac)-activated kinase 2 (PAK2) Copy number increase Neuronal differentiation Plausible 3q29 microdeletion syndrome 18, 75
Peripheral myelin protein 2 (PMP2) Copy number increase Myelin stabilization/Protection from demyelination Plausible Charcot-Marie-Tooth peroneal muscular atrophy 18, 76
Phosphodiesterase 4D interacting protein (PDE4DIP) Copy number increase Higher brain function Plausible Myeloproliferative disorder associated with eosinophilia 18, 40, 77
Protocadherin 11 X Y linked (PCDH11XY) Copy number increase/Expression change Cerebral asymmetry/Language development, neuroendocrine transdifferentiation Likely Klinefelter’s syndrome, Alzheimer’s disease, prostate cancer 18, 7882
Sialic acid-binding Ig superfamily lectin 5 (SIGLEC5) Expression change T-cell hyperactivation due to lowered expression Likely Susceptibility to T-cell mediated disease 64, 83
Sialic acid-binding Ig superfamily lectin 6 (SIGLEC6) Expression change Prolonged labor Plausible Preeclampsia 84
Sialic acid-binding Ig superfamily lectin 11 (SIGLEC11) Gene conversion/Expression change Alleviation of neurotoxicity from activated microglia. Potential neurotrophic effects. Likely Unknown 85, 86
Sialic acid-binding Ig superfamily lectin 13 (SIGLEC13) Gene loss Disease resistance to Sialylated Bacteria Likely Unknown 87
Solute carrier family 2 (facilitated glucose transporter) member 1 (SLC2A1) Expression change Metabolic changes in brain and skeletal muscle/Brain size Plausible GLUT1 deficiency syndrome 1 and 2, susceptibility to HTLV infection 88
Solute carrier family 2 (facilitated glucose transporter) member 4 (SLC2A4) Expression change Metabolic changes in brain and skeletal muscle/Brain size Plausible Noninsulin-dependent diabetes mellitus 88
Solute carrier family 6 (facilitated glucose transporter) member 13 (SLC6A13) Copy number increase Higher brain function Plausible Unknown 18, 89
Survival of motor neurone2, centromeric (SMN2) Novel gene variant Motor neuron maintenance, neuronal growth Likely Spinal muscular atrophy severity 18, 56
SLIT-ROBO Rho GTPase activating protein 2 (SRGAP2) Copy number increase Increased neuronal branching Likely Early infantile epileptic encephalopathy 18, 20, 25, 40
Sperm protein associated with the nucleus, X-linked family members A/D (SPANXA/D) Copy number increase Post-meiotic spermatogenesis Likely Unknown 90, 91
Thrombospondin 4 (THBS4) Expression change Synaptic organization and plasticity Plausible Familial premature coronary heart disease 92