Norio Niikawa

A strong association of axillary osmidrosis with the wet earwax type determined by genotyping of the ABCC11gene (2009)

Nakano, Motoi, Miwa, Nobutomo, Hirano, Akiyoshi, Yoshiura, Koh-ichiro, Niikawa, Norio

Abstract Background Two types of cerumen occur in humans: the wet type with brownish, sticky earwax, and the dry type with a lack of or reduced ceruminous secretion. The wet type is common in...

Novel deletion spanning RCC1-like domain of RPGR in Japanese X-linked retinitis pigmentosa family (2005)

Liu, Xiao-Qiang, Uchida, Asuka, Morishita, Kazuhiro, Hayakawa, Mutsuko, ...

PURPOSE: To describe a macrodeletion spanning entire RCC1-like doman in the RPGR gene in one Japanese family with X-linked retinitis pigmentosa (XLRP). METHODS: Clinical ophthalmologic examinations...

Neuron-specific relaxation of Igf2r imprinting is associated with neuron-specific histone modifications and lack of its antisense transcript Air (2005)

Yamasaki, Yoko, Kayashima, Tomohiko, Soejima, Hidenobu, Kinoshita, Akira, Yoshiura, Ko-ichiro, Matsumoto, Naomichi, ...

The mouse insulin-like growth factor II receptor (Igf2r) gene and its antisense transcript Air are reciprocally imprinted in most tissues, but in the brain, Igf2r is biallelically expressed despite...

Sotos syndrome common deletion is mediated by directly oriented subunits within inverted Sos-REP low-copy repeats (2005)

Kurotaki, Naohiro, Stankiewicz, Pawel, Wakui, Keiko, Niikawa, Norio, Lupski, James R.

Sotos syndrome (Sos) is an overgrowth disorder also characterized clinically by mental retardation, specific craniofacial features and advanced bone age. As NSD1 haploinsufficiency was determined in...

Sotos syndrome common deletion is mediated by directly oriented subunits within inverted Sos-REP low-copy repeats (2005)

Kurotaki, Naohiro, Stankiewicz, Pawel, Wakui, Keiko, Niikawa, Norio, Lupski, James R.

Sotos syndrome (Sos) is an overgrowth disorder also characterized clinically by mental retardation, specific craniofacial features, and advanced bone age. Since NSD1 haploinsufficiency was determined...

Neuron-specific relaxation of Igf2r imprinting is associated with neuron-specific histone modifications and lack of its antisense transcript Air (2005)

Yamasaki, Yoko, Kayashima, Tomohiko, Soejima, Hidenobu, Kinoshita, Akira, Yoshiura, Ko-ichiro, Matsumoto, Naomichi, ...

The mouse Igf2r gene and its antisense transcript Air are reciprocally imprinted in most tissues, but in the brain, Igf2r is biallelically expressed despite imprinted Air expression. To investigate...

Heterozygous TGFBR2 mutations in Marfan syndrome. (2004)

Mizuguchi, Takeshi, Collod-Beroud, Gwenaëlle, Akiyama, Takushi, Abifadel, Marianne, Harada, Naoki, Morisaki, Takayuki, ...

Marfan syndrome is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems associated with defects in the gene encoding fibrillin (FBN1) at...

Heterozygous TGFBR2 mutations in Marfan syndrome. (2004)

Mizuguchi, Takeshi, Collod-Beroud, Gwenaëlle, Akiyama, Takushi, Abifadel, Marianne, Harada, Naoki, Morisaki, Takayuki, ...

Marfan syndrome is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems associated with defects in the gene encoding fibrillin (FBN1) at...

Heterozygous TGFBR2 mutations in Marfan syndrome. (2004)

Mizuguchi, Takeshi, Collod-Beroud, Gwenaëlle, Akiyama, Takushi, Abifadel, Marianne, Harada, Naoki, Morisaki, Takayuki, ...

Marfan syndrome is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems associated with defects in the gene encoding fibrillin (FBN1) at...

LIT1, an imprinted antisense RNA in the human KvLQT1 locus identified by screening for differentially expressed transcripts using monochromosomal hybrids (1999)

Mitsuya, Kohzoh, Meguro, Makiko, Lee, Maxwell P., Katoh, Motonobu, Schulz, Thomas C., Kugoh, Hiroyuki, ...

Mammalian imprinted genes are frequently arranged in clusters on particular chromosomes. The imprinting cluster on human chromosome 11p15 is associated with Beckwith–Wiedemann syndrome (BWS) and a...

Characterization of the Promoter Region, First Ten Exons and Nine Intron-exon Boundaries of the DNA-dependent Protein Kinase Catalytic Subunit Gene, DNA-PKcs (XRCC7) (1997)

Fujimoto, Masahiro, Matsumoto, Naomichi, Tsujita, Takahiro, Tomita, Hiroaki, Kondo, Shinji, Miyake, Noriko, ...

The gene, DNAPKcs (XRCC7), for the human DNA-dependent protein kinase catalytic subunit (DNAPKcs) is a strong candidate that complements a severe combined immunodeciency (scid) and hypersensitivity...

Isolation and characterization of a novel gene deleted in DiGeorge syndrome (1995)

Kurahashi, Hiroki, Akagi, Kenzo, Inazawa, Johji, Ohta, Tohru, Niikawa, Norio, Kayatani, Futoshi, ...

The region commonly deleted in DiGeorge syndrome (DGS) has been localized at 22q11.1–q11.2 with the aid of a high resolution banding technique. A 22q11 specific plasmid library was constructed with...

cDNA Cloning, Expression, and Chromosomal Localization of Human N-Acetylglucosaminyltransferase III (GnT-III) (1993)

Ihara, Yoshito, Nishikawa, Atsushi, Tohma, Takaya, Soejima, Hidenobu, Niikawa, Norio, Taniguchi, Naoyuki

UDP-N-acetylglucosamine:β-D-mannoside β1,4 N-acetylglucosaminyltransferase III (GnT-III) [EC 2.4.1.144] catalyzes the addition of N-acetylglucosamine in β1–4 linkage to the β-linked mannose of...

A Simple and Efficient Amplification Method of DNA with Unknown Sequences and Its Application to Microdissection/Microcloning (1992)

Jinno, Yoshlhlro, Harada, Naoki, Yoshiura, Koh-ichiro, Ohta, Tohru, Tohma, Takaya, Hirota, Tetsuya, ...

An alternative method for amplification of DNA with unknown sequences was developed. This involves the direct ligation of a primer oligodeoxyribonucleotide itself to restricted DNA fragments with...

Cloning and characterization of the inversion breakpoint at chromosome 2q35 in a patient with Waardenburg syndrome type I (1992)

Tsukamoto, Kazuhiro, Tohma, Takaya, Ohta, Tohru, Yamakawa, Kazuhiro, Fukushima, Yoshimitsu, Nakamura, Yusuke, ...

We described cloning and characterization of an inversion breakpoint of chromosome 2 inv(2)(q35q37.3) observed in a patient with Waardenbrug syndrome type I (WSI) (1). Genomic cosmid clones...

Identification of proacrosin binding protein sp32 precursor as a human cancer/testis antigen

Ono, Toshiro, Kurashige, Takushi, Harada, Naoki, Noguchi, Yuji, Saika, Takashi, Niikawa, Norio, ...

Serological expression cloning of antigens eliciting a humoral immune response to a syngeneic mouse sarcoma identified pem (mouse placenta and embryonic expression gene) as a new member of the...

Genetic Mapping of the Camurati-Engelmann Disease Locus to Chromosome 19q13.1-q13.3

Ghadami, Mohsen, Makita, Yoshio, Yoshida, Kunihiro, Nishimura, Gen, Fukushima, Yoshimitsu, Wakui, Keiko, ...

Camurati-Engelmann disease (CED [MIM 131300]), or progressive diaphyseal dysplasia, is an autosomal dominant sclerosing bone dysplasia characterized by progressive bone formation along the periosteal...

Paroxysmal Kinesigenic Choreoathetosis Locus Maps to Chromosome 16p11.2-q12.1

Tomita, Hiro-aki, Nagamitsu, Shinichiro, Wakui, Keiko, Fukushima, Yoshimitsu, Yamada, Koki, Sadamatsu, Miyuki, ...

Paroxysmal kinesigenic choreoathetosis (PKC), the most frequently described type of paroxysmal dyskinesia, is characterized by recurrent, brief attacks of involuntary movements induced by sudden...

Identification of proacrosin binding protein sp32 precursor as a human cancer/testis antigen

Ono, Toshiro, Kurashige, Takushi, Harada, Naoki, Noguchi, Yuji, Saika, Takashi, Niikawa, Norio, ...

Serological expression cloning of antigens eliciting a humoral immune response to a syngeneic mouse sarcoma identified pem (mouse placenta and embryonic expression gene) as a new member of the...

Preferential Paternal Origin of Microdeletions Caused by Prezygotic Chromosome or Chromatid Rearrangements in Sotos Syndrome

Miyake, Noriko, Kurotaki, Naohiro, Sugawara, Hirobumi, Shimokawa, Osamu, Harada, Naoki, Kondoh, Tatsuro, ...

Sotos syndrome (SoS) is characterized by pre- and postnatal overgrowth with advanced bone age; a dysmorphic face with macrocephaly and pointed chin; large hands and feet; mental retardation; and...

Loss of CpG Methylation Is Strongly Correlated with Loss of Histone H3 Lysine 9 Methylation at DMR-LIT1 in Patients with Beckwith-Wiedemann Syndrome

Higashimoto, Ken, Urano, Takeshi, Sugiura, Kazumitsu, Yatsuki, Hitomi, Joh, Keiichiro, Zhao, Wei, ...

To clarify the chromatin-based imprinting mechanism of the p57KIP2/LIT1 subdomain at chromosome 11p15.5 and the mouse ortholog at chromosome 7F5, we investigated the histone-modification status at a...

Identification of a 3.0-kb Major Recombination Hotspot in Patients with Sotos Syndrome Who Carry a Common 1.9-Mb Microdeletion

Visser, Remco, Shimokawa, Osamu, Harada, Naoki, Kinoshita, Akira, Ohta, Tohru, Niikawa, Norio, ...

Sotos syndrome (SoS) is a congenital dysmorphic disorder characterized by overgrowth in childhood, distinctive craniofacial features, and mental retardation. Haploinsufficiency of the NSD1 gene owing...

Genetic Mapping of the Camurati-Engelmann Disease Locus to Chromosome 19q13.1-q13.3

Ghadami, Mohsen, Makita, Yoshio, Yoshida, Kunihiro, Nishimura, Gen, Fukushima, Yoshimitsu, Wakui, Keiko, ...

Camurati-Engelmann disease (CED [MIM 131300]), or progressive diaphyseal dysplasia, is an autosomal dominant sclerosing bone dysplasia characterized by progressive bone formation along the periosteal...

Paroxysmal Kinesigenic Choreoathetosis Locus Maps to Chromosome 16p11.2-q12.1

Tomita, Hiro-aki, Nagamitsu, Shinichiro, Wakui, Keiko, Fukushima, Yoshimitsu, Yamada, Koki, Sadamatsu, Miyuki, ...

Paroxysmal kinesigenic choreoathetosis (PKC), the most frequently described type of paroxysmal dyskinesia, is characterized by recurrent, brief attacks of involuntary movements induced by sudden...

Role of DNA Methylation and Histone H3 Lysine 27 Methylation in Tissue-Specific Imprinting of Mouse Grb10▿

Yamasaki-Ishizaki, Yoko, Kayashima, Tomohiko, Mapendano, Christophe K., Soejima, Hidenobu, Ohta, Tohru, Masuzaki, Hideaki, ...

Mouse Grb10 is a tissue-specific imprinted gene with promoter-specific expression. In most tissues, Grb10 is expressed exclusively from the major-type promoter of the maternal allele, whereas in the...