bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sleep in Women
Authors: Lee-Chiong Teofilo 
Published: November 2023

Abstract
Sleep can be affected by changes in reproductive cycles across the lifespan of women. Levels of estrogen and progesterone vary throughout the reproductive years, with a monthly cyclic pattern associated with menstruation, increasing during pregnancy, returning to preconception levels after delivery, and declining during perimenopause and menopause. Compared with men, women generally have more subjective complaints of insufficient or nonrestorative sleep while reporting a greater need for sleep.
The usual 28to 30-day menstrual cycle is divided into two phases, each lasting about 14 to 15 days. The initial follicular phase starts on the first day of menses. Estrogen is the predominant hormone during this phase. The second luteal phase begins at ovulation, with the peak of the surge in levels of luteinizing hormone brought about by rising levels of estrogen and progesterone. The subsequent menstruation is preceded by withdrawal of estrogen and progesterone. Mean daily body temperatures are lower by about 0.4°C during the follicular phase when compared with the luteal phase. Regular menstrual cycles start at menarche and end at menopause.
Medications and Their Effects on Sleep
Authors: Lee-Chiong Teofilo 
Published: November 2023

Abstract
Many medications affect the central nervous system (CNS) and can alter the patterns of sleep and wakefulness. Others can induce sleep disorders (eg, nightmares, periodic limb movements of sleep or restless legs syndrome) or exacerbate them (eg, obstructive sleep apnea). Some agents can cause both insomnia and excessive sleepiness in different individuals, or in the same individual under different circumstances. These can be a primary therapeutic effect of the drug, a withdrawal effect, or an adverse reaction. Each medication possesses class and agent-specific effects on sleep.
Alcohol’s effect on arousal and sleep is biphasic. Alcohol has both stimulatory effects (at low doses and on the rising phase of alcohol levels) and sedative effects (at high doses and on the falling phase of alcohol levels). Metabolism of alcohol occurs at a constant rate of 10 to 20 mg/dl per hour.
Alcohol’s sedating effect can be additive with other agents such as benzodiazepines. Acute ingestion decreases sleep latency, increases non–rapid eye movement (NREM) stages 3 and 4 sleep, and reduces rapid eye movement (REM) sleep during the first half of the sleep period (Table 16–1). It is metabolized quickly, and alcohol causes a rebound increase in the frequency of arousals and awakenings and an increase in REM sleep during the second part of the night. Nightmares and vivid dreams can occur. In addition, alcohol ingestion can cause worsening of snoring and obstructive sleep apnea by increasing upper airway resistance due to a decrease in pharyngeal dilator muscle tone.
Developmental Origins of the Mammalian Body Plan
Authors: Villar Angela J , Ware Stephanie M 
Published: November 2023

Abstract
The concept of the body plan refers to the distinguishing features of our bodies as organized three-dimensional structures. All members of a particular phylum share the same body plan at some point of development, whether it be at the embryonic, larval, or adult stage. Similarities and differences in adult shape and form are the basis of phylogenetic systematics. Organisms may be asymmetrical (no symmetry) or symmetrical (a single line or plane of symmetry). Like other vertebrates, mammals are bilaterally symmetrical quadrupeds and their close evolutionary relatives are readily distinguished from more distantly related organisms, such as sea urchins with spherical symmetry, sea anemones with radial symmetry and star7sh with 5-fold symmetry. Bilateral symmetry is a critical prerequisite for a moving, directional existence, the formation of a central nerve center and development of the head.
Development of the Nervous System
Authors: Rubenstein John L R , Puelles Luis 
Published: November 2023

Abstract
The nervous system has peripheral and central parts. The peripheral nervous system (PNS) is derived from subsets of neural crest cells (see Chapter 6) and from ectodermal placodes (e.g., olfactory and otic). PNS structures are organized as ganglia (e.g., sympathetic, parasympathetic, dorsal root, and enteric) or sensory epithelia (e.g., olfactory and otic; see Chapter 7). The central nervous system (CNS) is derived from the neural plate. CNS structures are categorized according to their location within the neural tube. This chapter focuses on a description of major steps that control the development of the CNS and how these steps can be derailed to cause birth defects. We will begin with a morphological description of CNS development (Rubenstein et al., 1998; Puelles, 2001).
Development of the Ear
Authors: Fekete Donna M 
Published: November 2023

Abstract
The inner ear subserves two distinct sensory systems: hearing and balance. The outer and middle ears are an integral part of hearing and so they must also be considered. Constructing the vertebrate ear poses special challenges because it is so anatomically complex and because there are multiple embryonic origins of the cells and tissues that comprise the ear. Coordination of these disparate cell populations necessarily involves inductive interactions and will probably include orchestrated feedback loops.
The Wnt Signaling Pathway
Authors: Kategaya Lorna S , Sheldahl, Laird C , Moon Randall T 
Published: November 2023

Abstract
The Wnt (wingless) signaling pathway is an important signal transduction pathway that is well conserved across animal phyla. Wnts are a large family of secreted signaling molecules known to play important and diverse roles in developmental processes such as cell fate speci7cation, cell migration, and cell proliferation. Misregulation of Wnt signaling can cause a range of developmental defects and is also implicated in the genesis of several human cancers in adults. While the 7rst Wnt was initially discovered as a protooncogene that leads to mammary tumor formation in mice, it was later shown to be important in axis formation in frogs (Nusse et al., 1991). In Drosophila, wingless (wg) had already been discovered, with mutants displaying segmentation defects or embryonic lethality (Nusslein-Volhard and Wieschaus, 1980).
NOG and Proximal Symphalangism (SYM1), Multiple Synostosis (SYN1), Tarsal–Carpal Coalition, and Isolated Stapes Ankylosis
Authors: Mundlos Stefan 
Published: November 2023

Abstract
Noggin (Nog) was first discovered as an important factor in brain development. The name reflects the observation that frog embryos injected with Nog mRNA grew exceptionally large heads. In Xenopus, Nog is able to mimic the activity of the Spemann organizer, which determines dorsal versus ventral fates in the gastrulating embryo. These and other studies demonstrated that Nog is a potent inhibitor of bone morphogenetic proteins (BMPs). Nog blocks the ability of BMPs to signal through cognate cell surface receptors by effectively masking the ligand-receptor–binding epitopes. Consequently, Nog has been widely used in experimental settings to negatively manipulate BMP signaling. Inactivation of Nog, on the other hand, results in excess BMP activity and, among many other developmental defects, in excess cartilage and a failure of joint formation. No human phenotype in association with a homozygous loss of function has been reported. However, four dominantly inherited conditions are associated with NOG mutations: proximal symphalangism (SYM1; OMIM 185800), multiple synostosis syndrome (SYN1; OMIM 186500), tarsal–carpal coalition (TCC; OMIM 186570), and isolated stapes ankylosis (OMIM 184460). All four conditions affect the development of the phalanges and/or joints resulting in brachydactyly and/or joint fusions. A second locus for proximal symphalangism (SYM2) has been shown to be associated with specific mutations in the gene encoding growth and differentiation factor 5 (GDF5).
ZEB2 and Mowat–Wilson Syndrome
Authors: Wilson Meredith , Goossens Michel , Moal, Florence Dastot-Le , Mowat David 
Published: November 2023

Abstract
Mowat–Wilson syndrome (MWS) (OMIM 235730) is a recently delineated mental retardation–multiple congenital anomaly syndrome characterized by typical facies, severe mental retardation (MR), epilepsy, and variable congenital malformations that include Hirschsprung disease (HSCR), congenital heart disease (CHD), urogenital anomalies, and agenesis of the corpus callosum (ACC).
Molecular and Cellular Biology of FGF Signaling
Authors: givol David , Eswarakumar veraragavan P 
Published: November 2023

Abstract
To date, 22 mammalian fibroblast growth factors (FGFs) are known. The first was discovered in the 1970s as a mitogen that can stimulate the growth of fibroblasts (Gospodarowicz, 1974). The FGFs range in size between 17 and 34 kDa in vertebrates, and they share a conserved core region of 120 amino acids that show 40%–60% identity. Their most important common property, besides binding to the FGF receptors (FGFRs), is the binding of heparan sulfate proteoglycan (HSPG) and high affinity for the glycosaminoglycan heparin (Burgess et al., 1989). This property served as the basis for methods to purify the various FGFs, and, as will be shown later, the binding of heparan sulfate (HS) is required for signaling by FGF (for review, see Ornitz et al., 2001). FGFs are present also in invertebrates. Drosophila has only one FGF (branchless), and Caenorhabditis elegans has two FGFs (egl-17 and let-756), suggesting extensive gene duplication during vertebrate development (Coulier et al., 1997).
Introduction to Endothelin-3/Endothelin-B Receptor and SOX10 Signaling Pathways
Authors: Gariepy Cheryl E 
Published: November 2023

Abstract
Hirschsprung disease [congenital absence of the distal enteric nervous system (ENS)] and Waardenburg syndrome (pigment abnormalities and sensorineural deafness) result from abnormal embryonic development of neural crest (NC)-derived cells. These developmental abnormalities occur together in humans (Shah–Waardenburg syndrome, also known as Waardenburg syndrome type IV) and in several species of animals. The Shah–Waardenburg syndrome is caused by mutations in the genes that encode the endothelin-B receptor (EDNRB), one of its ligands (endothelin-3, EDN3), and the Sex-determining Region Y (SRY)-related high mobility group (HMG)-box gene 10 (SOX10) (see Chapter 54). EDNRB and SOX10 are both critical components of a signaling cascade that controls the development of melanocytes and the ENS. Despite the similarities of the skin and enteric phenotypes observed in EDN3, EDNRB, and SOX10 mutant animals and humans, the interaction of these pathways in vivo is not yet fully de7ned, although some recent data suggest that SOX10 may regulate EDNRB expression in a tissueand developmental-stage–speci7c manner (Zhu et al., 2004; Hakami et al., 2006; Yokoyama et al., 2006).
The Role of Hox and Dlx Gene Clusters in Evolution and Development
Authors: Ruddle Frank h. 
Published: November 2023

Abstract
The genetic basis of development was significantly advanced in the early 1980s with the elucidation of the molecular structure of the homeotic genes. These genes constituted a large family characterized by a conserved 183-nucleotide (nt) domain termed the “homeobox” (Gehring, 1998). The homeobox encodes a 61-amino acid helix– turn–helix configuration that serves as a DNA-binding domain. The gene products of the homeotic genes serve as transcription factors and have been shown to regulate a variety of developmental processes, particularly in early development but at other stages in the lifecycle as well.
15q11-13 and the Prader–Willi Syndrome
Authors: Mccandless Shawn E , Cassidy Suzanne B 
Published: November 2023

Abstract
Prader–Willi syndrome [PWS (OMIM 176270)] is a multisystem disorder with a recognizable pattern of dysmorphic features and major neurologic, cognitive, endocrine, and behavioral abnormalities, all of which are referable to a characteristic pattern of central nervous system (CNS) maldevelopment. Newborns have profound hypotonia, feeding difficulties, and poor weight gain. Later, they develop hyperphagia usually leading to obesity, short stature, characteristic physical appearance, and a typical pattern of behavior problems that includes obsessive–compulsive traits. Management centers on control of food intake to avoid obesity, medical treatment of endocrine abnormalities, special educational programming, and behavior management techniques. Diagnosis is based on clinical findings, confirmed by demonstration of lack of biparental expression of genes in the PWS critical region on chromosome 15q11-q13.
NIPBL and SMC1L1 (now SCM1A) and the Cornelia de Lange Syndrome
Authors: Deardorff Matthew A , Krantz Ian D 
Published: November 2023

Abstract
Cornelia de Lange syndrome (CdLS) or Brachmann-de Lange syndrome (BDLS) (OMIM 122470, 300590 and 610759) is a dominant disorder of multiple congenital anomalies typically including characteristic facial features, hirsutism, abnormalities of the upper extremities, gastroesophageal dysfunction, growth retardation, and neurodevelopmental delay. Mutations in genes encoding components and regulators of the cohesin complex (to date NIPBL and SMC1L1) are causative.
GATA3 AND HYPOPARATHRYOIDISM, DEAFNESS AND RENAL DISEASE
Authors: Thakker R V 
Published: November 2023

Abstract
GATA3 haploinsufficiency is associated with the hypoparathyroidism, deafness and renal dysplasia (HDR) syndrome (van Esch et al., 2000). This chapter will brie8y review: the GATA family members and their role in vertebrate embryonic development with an emphasis on GATA3, the phenotype of HDR, the GATA3 mutations associated with HDR, and the phenotype of a mouse model that is deleted for Gata3.
GLMN and Glomuvenous Malformation
Authors: Brouillard Pascal , Enjolras Odile , Boon, Laurence M , Vikkula Miikka 
Published: November 2023

Abstract
Glomuvenous malformation (GVM) is a localized cutaneous vascular lesion histologically characterized by the presence of maldifferentiated smooth muscle-like “glomus cells” around distended vein-like channels. GVM is caused by mutations in a gene named Glomulin (GLMN). As most mutations cause premature termination codons, they probably result in loss-of-function. Paradominant inheritance was suggested by the identification of a double-hit mutation in one lesion, indicating that GVM lesions may be due to complete localized loss of glomulin. Glomulin is specifically expressed in vascular smooth muscle cells (vSMCs), but its exact function remains unknown. It likely plays a role in the differentiation of vSMCs, as in GVM these cells have deviated in their differentiation process due to lack of glomulin expression. The transforming growth factor β (TGF-β) and hepatocyte growth factor (HGF) signaling pathways may be altered.
The Williamsburg Circle
Authors: Hayes Kevin J 
Published: November 2023

Abstract
Thomas Jefferson left William and Mary on April 25, 1762, without taking a degree. Under the old requirements, he had already attended college long enough to earn his baccalaureate. Under the new ones, he was only half finished. He saw no reason to continue. Richard Graham, dismissed as Professor of Natural Philosophy three years earlier, had been reappointed to the faculty in mid-1761, this time as Professor of Moral Philosophy. Despite Graham’s learning—he had an M.A. from Oxford—he made little impression on Jefferson, who much preferred William Small as Professor of Moral Philosophy. Deciding to leave college, Jefferson saw that getting an education was more important than earning a degree. He believed that a good education extends beyond the walls of a classroom.
Becoming a Burgess
Authors: Hayes Kevin J 
Published: November 2023

Abstract
Since starting college, Jefferson had been traveling back and forth from Shadwell to Williamsburg at least twice a year. To him Williamsburg was not only the place where he received his professional education, but also a place where he learned the social graces and developed his love of science and literature through the conviviality of friends he made there. Arriving in town a week before the spring session of General Court was scheduled to convene in 1768, he found his familiar Williamsburg world irreparably changed: his dear friend Lieutenant Governor Francis Fauquier had passed away the previous month. His death meant not only the end of the pleasant intellectual conversation around the dinner table at the Governor’s Palace that Jefferson so enjoyed, but also the end of their ensemble musical performances. Even after his death, Fauquier still contributed to the advancement of scientific inquiry in Virginia: his will stipulated that in case he died of undetermined causes, his body should be used for purposes of scientific research.
A Summary View of the Rights of British America
Authors: Hayes Kevin J 
Published: November 2023

Abstract
In 1773, Virginia welcomed one of the most colorful characters yet to reach its shores, the Florentine viticulturist Philip Mazzei, whose personal memoirs provide a unique glimpse into Jefferson’s world, despite their occasional errors. With an eye for detail and an ear for dialogue, Mazzei brought his Virginia experience alive as he wrote his life story. Having left Tuscany the previous decade, he had been living in London, where he befriended many Americans, including Thomas Adams and Benjamin Franklin. Adams came to know Mazzei well and spoke with him at length about Virginia and its leading citizens. Conversing with Mazzei—a great talker—people sometimes found it difficult to get a word in edgewise, but Adams managed to tell him about Thomas Jefferson, whose presence in the colony confirmed that Virginia was not without men of refinement and intellect. Before long, Mazzei—a great dreamer as well as a great talker—made up his mind to immigrate to Virginia, where he hoped to establish vineyards and olive orchards and start producing wine and olive oil on a grand scale. From England, he returned to Tuscany to prepare for his voyage to the New World.
London Town
Authors: Hayes Kevin J 
Published: November 2023

Abstract
One snowy February day in 1786, Jefferson received a visit from William Stephens Smith, secretary of the American legation in London, good friend of John Adams, and fiancéto his daughter Abigail. Smith had come to Paris expressly to deliver an urgent message from Adams to Jefferson. Since their joint commission to negotiate treaties of amity and commerce between the United States and other nations would end in May, the two had the responsibility to do as much as they could before its expiration. Though any further efforts in this regard had seemed fruitless, new and unexpected opportunities for negotiating treaties with Portugal, Tripoli, and possibly other nations of the Barbary Coast arose that winter. In recent weeks, Adams had been meeting with Abdrahaman, the envoy of the Sultan of Tripoli.1 Adams went so far to endear himself to the Tripoline ambassador that he actually began smoking a hookah in his presence. The incongruous sight of this staid New Englander puffing away at a hookah prompted Abdrahaman’s secretary to exclaim, “Monsieur, vouseêtes un Turk!”
An Inquisitive Journey through France and Italy
Authors: Hayes Kevin J 
Published: November 2023

Abstract
Jefferson’s broken wrist healed very slowly and he considered more drastic treatment. Friends recommended the waters at Aix-enProvence for their curative properties. Chances are he had little faith in the waters at Aix, but the promised cure gave him a convenient excuse to visit Provence. He had other ways to justify the trip, which would let him visit Italy, a place he had dreamt of seeing for years. Despite what the South of France and the Italian north offered visitors in terms of art and architecture, Jefferson really wanted to observe the agriculture of these regions and the folkways of their people. The story of this European tour has been told before. What has so far gone untold is the story behind the story. Jefferson wrote different versions of his journey for different purposes.
The Young Idea
Authors: Hayes Kevin J 
Published: November 2023

Abstract
Left Paris”: So reads Jefferson’s memorandum book for Saturday, September 26, 1789. Two days later his party, which included his two daughters, James Hemings, and James’s sister Sally, reached Le Havre, where Jefferson befriended a seafaring man from Massachusetts named Nathaniel Cutting. Better known to posterity as a diarist than a sailor, Captain Cutting tirelessly recorded the incidents of everyday life to create a lasting record of his seafaring adventures. Cutting’s diary chronicles his friendship with the Jeffersons in great detail and thus supplements the laconic memorandum book. Few surviving documents better capture Jefferson’s personal interactions with his daughters. Since it took several days to find passage from Le Havre to the Isle of Wight, Cutting spent much time in their company, postponing his own journey to be with them.
Return to Monticello
Authors: Hayes Kevin J 
Published: November 2023

Abstract
During his presidency, Thomas Jefferson had accumulated a considerable number of personal possessions, including, of course, lots and lots of books. Anticipating his retirement by more than a year, he had sent much of his presidential library back to Monticello, enough books to fill eight trunks. Together with four other packages, these bookfilled trunks weighed two and a half tons. As his retirement approached in February 1809, enough other stuff remained—books too essential to pack, papers, furniture, lamps—that he summoned overseer Edmund Bacon to come up from Monticello to help. Together they filled about thirty packages to be sent home by water. The rest they loaded onto wagons driven to Washington. Bacon recalled, “I had three wagons from Monticello—two six-mule teams loaded with boxes, and the other four sorrel Chickasaw horses, and the wagon pretty much loaded with shrubbery from Maine’s nursery. The servants rode on these wagons. I had the carriage horses and carriage, and rode behind them.” Bacon left Washington on March ninth or tenth. Jefferson followed on Saturday, the eleventh.
Compression of the Anterior Visual Pathways
Authors: Foroozan Rod , Hinckley Lisa 
Published: November 2023

Abstract
Unilateral or bilateral visual loss may occur from a compressive process affecting the intraorbital optic nerves, intracranial optic nerves, optic chiasm, or optic tracts. The term compressive, as used in this chapter, includes benign and malignant neoplasm, cyst, mucocele, and aneurysm. Also included is the orbital compression of the optic nerves by enlarged extraocular muscles in thyroid-associated orbitopathy.
Toxic and Nutritional Optic Neuropathy
Authors: Kerrison John. B 
Published: November 2023

Abstract
Although the clinical profiles of patients with toxic optic neuropathy differ from the profiles of those with nutritional optic neuropathy, the pattern of visual loss is similar. It is characterized by decreased visual acuity with relative preservation of peripheral vision. Findings on examination are that of a bilateral optic neuropathy. Visual acuity characteristically ranges from 20/30–20/200. Significant dyschromatopsia is present, often disproportionate to the level of visual acuity.