bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Law of Peoples, Social Cooperation, Human Rights, and Distributive Justice
Authors: Freeman Samuel 
Published: November 2023

Abstract
I. Introduction
My aim in this essay is to discuss, and defend against some frequent objections, John Rawls’s rejection of a global principle of distributive justice. As is well known, Rawls’s A Theory of Justice argues for a principle of distributive justice, the difference principle, that is to be applied within different societies but not among them.1 According to A Theory of Justice, each society has the duty to set up its economic and legal institutions in such a way that they make the least advantaged among its own members better off than the least advantaged would be if that society were structured according to any other distribution principle. But each society does not have a duty to structure its system so as to maximize the position of the least advantaged in the world at large. Though it is a universal principle that is to apply severally, or within every society, the difference principle is not global in reach, applying jointly to all societies simultaneously. To critics of many political persuasions, this seems a peculiar position. Why should principles of justice be domestically rather than globally applied?
Rawls’s position in A Theory of Justice becomes even more complicated in Political Liberalism and The Law of Peoples, where he is guided by questions of political legitimacy and the feasibility (or “stability”) of liberal regimes.2 In Political Liberalism and later works,3 Rawls appears to give up on the idea that a well-
There Are No Ordinary Things
Authors: Unger Peter 
Published: November 2023

Abstract
Human experience, it may be said, naturally leads us to have a certain view ofreality, which I call the view of common sense. This view is tempered by cultural advance, but in basic form it is similar for all cultures on this planet, even the most primitive and isolated. According to this prevalent view, there are various sorts of ordinary things in the world. Some of these are made by man, such as tables and chairs and spears, and in some ‘advanced’ cultures also swizzle sticks and sousaphones. Some are found in nature such as stones and rocks and twigs, and also tumbleweeds and fingernails. I believe that none of these things exist, and so that the view of common sense is badly in error. In this paper, I shall argue for this negative belief of mine.
The Mental Problems Of The Many
Authors: Unger Peter 
Published: November 2023

Abstract
Many years ago, I blush to recall, I published some arguments against the existence of all sorts of commonly supposed entities-against rocks and desks, plants and planets, stars and salt shakers, human brains and bodies, and, perish the thought, against us human thinking experiencers, including even the one who’s me. By contrast, now I’m trying to develop, in a book I’ve been long writing, a humanly realistic philosophy, wherein my existence, and yours, has the status of a quite undeniable philosophic datum. As it seems to me now, certain trying ideas then deployed in such nihilistic reasoning may bear importantly on the question of what sort of a humanly realistic view we should adopt. These are ideas to the effect that, where I’m apt first to think that there’s just this one human body, “my body”, seated in just this one chair, “my desk chair”, there are, more accurately, many billions of human bodies, each seated in many billions of chairs. And, where I’m first given to believe that there’s just one healthy active brain, “my brain”, promoting someone’s mentality, there may be many billions of brains, each of them largely overlapping so many of the others, and each serving, quite equally, to promote a thinking, experiencing and choosing human being, or human self. Maybe each brain promotes the very same mind, or self, as do each of the others, in which case there’s just one self promoted (rather redundantly?) by them all; or maybe each promotes a numerically distinct conscious individual, in which case many billions of experiencers may be, in my situation, simultaneously promoted. Right now, these remarks should seem no better than cryptic comments; but, in the course of this essay their import should become clearer.
Précis Of Identity, Consciousness And Value
Authors: Unger Peter 
Published: November 2023

Abstract
While it concerns other large issues as well, a single question focuses the inquiry of this book1: What is involved when an actual person, like me, survives until some future time, like a century from now? To this question, some seek a completely general answer. Even if Berkeley should be right and there is no physical reality, their (desired) answer is to inform us about our survival. Although pretty ambitious in the matter, I’m content to settle for a lot less than that. For me, it’s enough to give an answer that articulates, along lines general enough to prove philosophically interesting, our deep beliefs about the conditions of our survival. There’s a good reason for this: By disclosing our deep beliefs about our survival, I indirectly articulate what are, as we most deeply believe, some quite general conditions of our survival. How do I detect these deep beliefs? Doing informal psychology, I uncover them by noting people’s responses to examples. But many examples have contexts where responses are misleading. And, even in revealing contexts, some examples promote responses that are best explained away, as arising from sources other than our deep beliefs about ourselves. Still, often, we may sensibly avoid all the pitfalls. In chapter 1, I describe and defend the aims and methods of the inquiry.
The Search for New Sources of Pluripotent Stem Cells
Authors: Cohen Cynthia B 
Published: November 2023

Abstract
The number of human embryonic stem cell lines currently available for research in the United States, some scientists, legislators, and commentators maintain, is insuffcient for research and therapeutic eVorts. They find that the policy of President George W. Bush of providing federal funding only for research on twenty-two embryonic stem cell lines derived before August 2001 has left researchers with too small a number of stem cell lines with which to go forward at a rapid pace. Furthermore, cells that are maintained in culture for a significant length of time tend to deteriorate, including those listed on the National Institutes of Health (NIH) registry. Many of these stem cell lines, although some say not all, have been cultured with nonhuman cells or serum that would pose health risks to patients who received differentiated offshoots from them in research or therapy. For such reasons, stem cell investigators are attempting to develop a larger pool of human pluripotent stem cells—cells that can transform into almost any cell of the body except placental tissue—by expanding the use of certain currently available sources of these cells and developing new sources.
International Stem Cell Research and Research Cloning: Three Contrasting Approaches
Authors: Cohen Cynthia B 
Published: November 2023

Abstract
Recent advances in stem cell research have led a surprising number of countries around the globe to grapple with the question of whether to engage in research on these novel cells. These countries have responded in different ways to the ethical and policy challenges posed by stem cell research, depending on their history, cultural traditions, prevalent ethical and religious values, degree of technological optimism, and attitudes toward economic development through innovative scientific research.
Some, galvanized by the strong scientific, therapeutic, ethical, and economic pull of this research, have resolved to pursue stem cell research vigorously. The United Kingdom, Japan, China, Israel, Singapore, and South Korea are among them. They have not only embraced both embryonic stem cell research and research cloning but also set out to become the premier centers of stem cell research in the world. However, they have not allowed such research to proceed willy-nilly. Instead, they have established guidelines and regulations to govern it. I term their approach to stem cell research a “permissive” one.
In Pursuit of National Review and Oversight of Stem Cell Research in the United States
Authors: Cohen Cynthia B 
Published: November 2023

Abstract
Stem cell research brings to the fore unique and fundamental ethical questions not only about whether to engage in research using early human embryos but also about a host of other issues that revolve around how we ought to use the regenerative powers that this research offers. These additional issues include such current questions as whether to employ some of the innovative techniques proposed for developing additional sources of human pluripotent stem cells, not only to treat those laid low by diseases, but also to bring babies into the world who would not have such diseases, and where to draw ethical boundaries around the transfer of human stem cells into nonhumans in the course of stem cell research designed to regenerate damaged human cells.
Development of Epidermal Appendages: Teeth and Hair
Authors: Ohazama Atsushi , Sharpe Paul T 
Published: November 2023

Abstract
The skin serves several functions, including thermoregulation, protection from the external environment, maintaining internal tissue Muid from evaporation, reduction of friction in water, sensation, defense against predation and infection, and supporting the hair and feathers. The distribution of pigment in skin and odors from the skin are also important for social interaction and defense. To ful7ll these multiple functions, the skin develops many structures as epidermal appendages (e.g., scales, feathers, hair, horns, nails, claws, sweat glands, sebaceous glands, and mammary glands). In addition, other epidermal appendages, most notably teeth, develop from the same ectoderm as skin in the oral cavity.
The Sonic Hedgehog Signaling Pathway
Authors: Iannaccone Philip , Holmgren Robert , Lamm Marilyn L G , Ahlgren Sara , Lakiza Olga , Yoon, Joon Won , Walterhouse David 
Published: November 2023

Abstract
The highly conserved hedgehog (Hh) family of signaling proteins triggers crucial processes in early vertebrate and invertebrate development, including cell fate speci7cation, patterning, and organogenesis. The development of a large number of organ systems is directly affected by the Hh signaling pathway. Indeed, the Hh ligands, patched and smoothened transmembrane proteins, and the Ci/Gli family of transcription factors are at the nexus of mesodermal development and they are critical to epithelial–mesenchymal interactions throughout the body. It is also becoming clear that a relatively small number of pathways, the Hh pathway included, operating in a combinatorial manner are responsible for pattern formation in early development. Genetic and epigenetic aberrations, pharmacological perturbations, and environmental insults that impact the Hh signaling pathway translate to various developmental disorders and diseases, including cancer (reviewed in Villavicencio et al., 2000; Cohen, 2003). The wide variety of diseases associated with the genes in this pathway usually involves the organs and tissues in which the pathway normally operates, including the brain, gastrointestinal tract, skin, prostate, ovary, lung, muscle, and bone. Although still incompletse, our understanding of the Hh pathway has progressed rapidly in the past few years, promising a future of accurate diagnosis and effective treatment of diseases related to defects in Hh signaling.
Several proteins cooperatively transduce an extracellular signal from the Hh ligand through a cascade of steps, the net result of which is a change in the pattern of expression of genes within the target cell. This change then coordinates and regulates proliferation and differentiation of various cells in a way that develops and maintains pattern. The main cast of characters in the story includes Drosophila Hh and the vertebrate homologs, Sonic hedgehog (Shh), Indian hedgehog (Ihh), and Desert hedgehog (Dhh); Drosophila Patched (Ptc) and the vertebrate homologs Ptc1 and Ptc2; Drosophila and vertebrate Smoothened (Smo); Drosophila Costal 2 (Cos2), vertebrate Kif family members, and yet unidenti7ed vertebrate homolog(s); Drosophila and vertebrate Fused (Fu); Drosophila and vertebrate Suppressor of fused [Su(fu)]; Drosophila Cubitus interruptus (Ci) and the vertebrate homologs, Gli1, Gli2, and Gli3; and a variety of interacting proteins and intracellular regulators. In this chapter, we will examine the components and function of the pathway in the Drosophila (fruit My) model system from where most of the seminal discoveries originate and in vertebrate systems, including mouse and human, which exhibit unique features.
ROR2 and Brachydactyly Type B and Recessive Robinow Syndrome
Authors: Wilkie Andrew O M 
Published: November 2023

Abstract
Rutations of the gene ROR2 (receptor tyrosine kinase-like orphan receptor 1), which maps to 9q22 and encodes a receptor tyrosine kinase (RTK) that participates in Wingless-type (Wnt) signaling, have been identi%ed in two distinct skeletal disorders, dominantly inherited brachydactyly type B (BDB) (OMIM 113000) and recessive Robinow syndrome (RRS). This demonstrates that ROR2 is required for the development of multiple organs, most prominently bone, but including, in the case of RRS, the face, heart, and urogenital system. A single unlinked paralogue Ror1/ROR1 is present in mice and humans, but mutations of ROR1 have not yet been identi%ed in any human disorder.
LEMD3 and Osteopoikilosis, the Buschke–Ollendorff Syndrome and Melorheostosis
Authors: Hellemans Jan , Mortier Geert R 
Published: November 2023

Abstract
LEMD3 is an integral protein of the inner nuclear membrane and has a molecular mass of approximately 100 kDa. It is composed of a nucleoplasmic N-terminal domain, a #rst transmembrane segment, a luminal loop, a second transmembrane segment and a nucleoplasmic C-terminal domain (Fig. 38–1) (Lin et al., 2000). LEMD3 was previously known as MAN1. It was initially identi#ed as a nuclear envelope protein recognized by autoantibodies from a patient (MAN refers to the initials of this patient) with an ill-de#ned collagen vascular disease (Paulin-Levasseur et al., 1996). To avoid confusion with the abbreviations of the different mannosidase enzymes, MAN1 was recently renamed as LEMD3 (LEM domain containing 3), herewith referring to the LEM domain in the N-terminal portion of the protein. The LEM domain is a conserved globular motif of approximately 40 amino acids found in several inner nuclear membrane and nucleoplasmic proteins. “LEM” refers to the three proteins in which the domain was initially characterized: LAP2, emerin, and MAN1.
TGFBR 1/2 and Loeys–Dietz Syndrome
Authors: Loeys Bart L , Dietz Harry C 
Published: November 2023

Abstract
A novel autosomal dominant aortic aneurysm syndrome characterized by the triad of hypertelorism, bifid uvula/cleft palate, and arterial tortuosity with ascending aortic aneurysm/dissection was initially described in 10 families [Loeys–Dietz syndrome (LDS); MIM 609192] (Fig. 39–1) (Loeys et al., 2005). This syndrome also presents with multiple other findings, including craniosynostosis, Arnold Chiari type I malformation, pectus deformity, scoliosis, arachnodactyly, club feet, patent ductus arteriosus, atrial septal defect, bicuspid semilunar valves, and aneurysms/dissections throughout the arterial tree.
FGF Receptor Mutations: Bone Dysplasia, Craniosynostosis, and Other Syndromes
Authors: Wilkie Andrew O M 
Published: November 2023

Abstract
Mutations in three of the four fibroblast growth factor receptors (FGFR1, FGFR2, and FGFR3) have been identified in congenital disorders with a very wide variety of phenotypes. Several features make these mutations of particular interest. The associated clinical conditions are, collectively, a common cause of monogenic disease; the mutation rates for several of these disorders are exceptionally high, and are beginning to reveal a novel mechanism of disease through the selection of spermatogonial mutations; FGFR mutations illustrate the close link between developmental disorders and cancer; and the diversity of phenotypes is re)ected in a diversity of mechanisms of molecular pathogenesis, making FGFR mutations a paradigm for understanding the many bases of genetic dominance. The mutant phenotypes have given important clues to the roles of FGFRs in the development of epiphyseal plate, cranial sutures, limb bud and olfactory neurons, amongst other structures. The accompanying chapter gives an introduction to FGFR structure and biology, which is not detailed here.
TEK (TIE2) and Cutaneomucosal Venous Malformation
Authors: wouters Vinciane , Boon laurence M , Mulliken, john B , vikkula miikka 
Published: November 2023

Abstract
Venous anomalies are the most frequent malformations referred to vascular anomalies centers. They are divided into venous malformations (VMs, 95%) and glomuvenous malformations (GVMs, 5%; see Chapter 183). VMs typically involve skin, mucosa, and subcutis, but they also arise in muscle, bones, and internal organs. Lesions can be solitary or multifocal; the latter presentation is likely to be an autosomal dominant familial trait, termed cutaneomucosal venous malformation (VMCM; MIM 600195). VMCM represents approximately 1% of VMs. VMCM is caused by amino acid substitutions in the tyrosine kinase domain of the angiopoietin receptor TEK (now known as TIE2, which is used in this chapter). These gain-of-function mutations increase receptor phosphorylation. VMs can also be part of complex vascular disorders of unknown etiology, for example, Klippel–Trenaunay syndrome, Maffucci syndrome, and Blue Rubber Bleb Nevus syndrome.
HRAS and Costello Syndrome
Authors: Aoki Yoko , Matsubara Yoichi 
Published: November 2023

Abstract
Costello syndrome (MIM 218040) is a multiple congenital anomaly/ mental retardation syndrome originally reported by J.M. Costello in 1971 (Costello, 1971). Patients with Costello syndrome show a coarse face, loose skin, hypermobility of the small joints, heart defects including cardiomyopathy, and predisposition to tumors. In 2005, germline mutations in the Harvey rat sarcoma (HRAS) proto-oncogene were identified in patients with Costello syndrome (Aoki et al., 2005). All patients had mutations in the heterozygous form, indicating autosomal dominant inheritance. Molecular analysis of parents showed that mutations were not inherited, suggesting mutations occurred de novo. Costello syndrome is the first disorder, which has been found to be associated with germline mutations in the RAS subfamily of small guanosine triphosphatases (GTPases). These observations suggest that the proto-oncogene HRAS plays an important role in human development.
HOXA11 and Amegakaryocytic Thrombocytopenia with Radioulnar Synostosis
Authors: Geddis Amy E 
Published: November 2023

Abstract
Congenital thrombocytopenia is associated with radial abnormalities in several disorders, although the genetic basis of this linkage remains poorly understood. These disorders include thrombocytopenia with absent radii (TAR), Fanconi’s anemia (FA), and amegakaryocytic thrombocytopenia with radioulnar synostosis (ATRUS). Although FA is now recognized to be a defect of DNA repair, the etiology of thrombocytopenia and radial defects in TAR and ATRUS remains unclear. The description of two families in which a heterozygous mutation of Homeobox A11 (HOXA11) was inherited with the phenotype of thrombocytopenia and radioulnar synostosis provided the (rst insights into the molecular mechanism of ATRUS.
PITX2 and PITX3 and the Axenfeld–Rieger Syndrome, Peters Anomaly, Anterior Segment Ocular Dysgenesis, Ring Dermoid of the Cornea, Posterior Polar Congenital Cataract (CPP4), and Microphthalmia with Neurologic Impairment
Authors: Semina Elena V 
Published: November 2023

Abstract
PITX2 and PITX3 represent members of the PITX family of homeobox-containing transcription factor genes that was shown to play an important role in embryonic development in different species. Humans with mutations in the PITX2 or PITX3 genes were found to exhibit a variety of ocular phenotypes such as glaucoma, cataracts, corneal anomalies, and microphthalmia that in many cases are accompanied by other developmental defects. In animal models, Pitx2 gene was shown to be essential to normal development of multiple organs such as eyes, teeth, brain, heart, lungs, liver, and body wall. Pitx3 gene was found to be involved in the development of lens and the mesencephalic dopaminergic system. Therefore, these PITX genes represent key components of the network of genes directing development of multiple organs in various species. Studies of the PITX roles in embryogenesis and cell fate decisions will lead to further exploration of the disorders associated with alterations in these genes. This will provide new insight into mechanisms of normal embryonic development, allow identification of other disease genes, and inevitably lead to the better diagnosis and treatment of the associated conditions.
LMX1B and the Nail Patella Syndrome
Authors: Morello Roy , Lennington, Jennifer N , Lee Brendan 
Published: November 2023

Abstract
Nail patella syndrome (NPS) is a dominantly inherited skeletal malformation syndrome. Patients have characteristic features including nail and patella hypoplasia, elbow and knee deformities, nephropathy, and ocular defects. The condition is characterized by high penetrance, variable expressivity, and signi)cant intrafamilial variability. Diagnosis is based on clinical criteria. Morbidity is associated with functional limitations caused by joint abnormalities, risk of developing nephrotic syndrome [which may progress to end-stage renal disease (ESRD)], and risk of developing glaucoma and ocular hypertension. NPS is caused by loss-of-function mutations in the LIM homeodomain (LHX) transcription factor LMX1B. LMX1B speci)es a transcriptional network important in pattern formation of the limb as well as cell maturation and differentiation in both the kidney and the central nervous system (CNS). In the limb, LMX1B is a unique mesenchymal determinant of dorsal cell fate. In the kidney, it regulates podocyte-speci)c expression of glomerular basement membrane (GBM) type IV collagen. Moreover, it speci)es morphogenesis of secondary foot processes and directs the expression of unique components of a specialized cell–cell adhesion complex, the slit diaphragm. Finally, it has important roles during CNS formation, including the survival of mesencephalic dopaminergic neurons, the development of all serotonergic neurons, and the differentiation of periocular mesenchyme. The pathogenesis of NPS re%ects a loss of dorsal speci)cation of soft tissues of the limb and disrupted proliferation of anterior relative to posterior skeletal elements. The nephropathy arises in part owing to an architectural defect in a primary component of the )ltration barrier of the kidney, the GBM. Glaucoma is likely secondary to anterior segment dysgenesis in the eye. How the function of LMX1B in other developmental programs impacts the pathogenesis of the NPS phenotype is still unclear.
PAX3 and Waardenburg Syndrome Type 1
Authors: Corry Gareth N , Underhill D Alan 
Published: November 2023

Abstract
The human PAX genes encode a group of sequence-specific transcription factors that play important roles in development and cell differentiation (Underhill, 2000; Chi and Epstein, 2002). Pax proteins are characterized by a 128–amino acid DNA-binding paired domain (PD) and are highly conserved in metazoans. Four of nine mammalian Pax proteins also contain a homeodomain (HD), and six are directly implicated in congenital syndromes or cancer in humans.