Characterization of the bone phenotype in ClC-7-deficient mice.


:Mice deficient in the chloride channel ClC-7, which is likely involved in acidification of the resorption lacuna, display severe osteopetrosis. To fully characterize the osteopetrotic phenotype, the phenotypes of osteoclasts and osteoblasts were evaluated. ClC-7(-/-) mice and their corresponding wild-type littermates were killed at 4-5 weeks of age. Biochemical markers of bone resorption (CTX-I), osteoclast number (TRAP5b), and osteoblast activity (ALP) were evaluated in serum. Splenocytes were differentiated into osteoclasts using M-CSF and RANKL. Mature osteoclasts were seeded on calcified or decalcified bone slices, and CTX-I, Ca(2+), and TRAP were measured. Acidification rates in membrane vesicles from bone cells were measured using acridine orange. Osteoblastogenesis and nodule formation in vitro were investigated using calvarial osteoblasts. ClC-7(-/-) osteoclasts were unable to resorb calcified bone in vitro. However, osteoclasts were able to degrade decalcified bone. Acid influx in bone membrane vesicles was reduced by 70% in ClC-7(-/-) mice. Serum ALP was increased by 30% and TRAP5b was increased by 250% in ClC-7(-/-) mice, whereas the CTX/TRAP5b ratio was reduced to 50% of the wild-type level. Finally, evaluation of calvarial ClC-7(-/-) osteoblasts showed normal osteoblastogenesis. In summary, we present evidence supporting a pivotal role for ClC-7 in acidification of the resorption lacuna and evidence indicating that bone formation and bone resorption are no longer balanced in ClC-7(-/-) mice.


Calcif Tissue Int


Neutzsky-Wulff AV,Karsdal MA,Henriksen K




Has Abstract


2008-12-01 00:00:00












  • Vitamin K1 supplementation retards bone loss in postmenopausal women between 50 and 60 years of age.

    abstract::Although several observational studies have demonstrated an association between vitamin K status and bone mineral density (BMD) in postmenopausal women, no placebo-controlled intervention trials of the effect of vitamin K1 supplementation on bone loss have been reported thus far. In the trial presented here we have in...

    journal_title:Calcified tissue international

    pub_type: 临床试验,杂志文章,随机对照试验


    authors: Braam LA,Knapen MH,Geusens P,Brouns F,Hamulyák K,Gerichhausen MJ,Vermeer C

    更新日期:2003-07-01 00:00:00

  • Fourier transform infrared microspectroscopic investigation of the organic and mineral constituents of peritubular dentin: a horse study.

    abstract::Peritubular dentin (PTD) is a relatively dense mineralized tissue surrounding tooth dentin tubules, whose composition and mode of formation are still unclear. Fourier transform infrared microspectroscopic studies of the organic and mineral components of the highly developed horse PTD indicate that the peritubular matr...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Magne D,Guicheux J,Weiss P,Pilet P,Daculsi G

    更新日期:2002-08-01 00:00:00

  • Point-of-Care Phalangeal Bone Mineral Density Measurement Can Reduce the Need of Dual-Energy X-Ray Absorptiometry Scanning in Danish Women at Risk of Fracture.

    abstract::Identifying persons with a high risk of osteoporotic fractures remains a challenge. DXA uptake in women with elevated risk of osteoporosis seems to be depending on distance to scanning facilities. This study aimed to investigate the ability of a small portable scanner in identifying women with reduced bone mineral den...

    journal_title:Calcified tissue international

    pub_type: 杂志文章,随机对照试验


    authors: Holmberg T,Bech M,Gram J,Hermann AP,Rubin KH,Brixen K

    更新日期:2016-03-01 00:00:00

  • Treatment thresholds for osteoporosis and reimbursability criteria: perspectives associated with fracture risk-assessment tools.

    abstract::The definition of osteoporosis was based for several years on bone mineral density values, which were used by most guidelines for defining treatment thresholds. The availability of tools for the estimation of fracture risk, such as FRAX™ or its adapted Italian version, DeFRA, is providing a way to grade osteoporosis s...

    journal_title:Calcified tissue international

    pub_type: 社论


    authors: Adami S,Bertoldo F,Gatti D,Minisola G,Rossini M,Sinigaglia L,Varenna M

    更新日期:2013-09-01 00:00:00

  • X-ray microanalysis of teeth from healthy patients and patients with familial hypophosphatemia.

    abstract::Energy-dispersive X-ray microanalysis was used to determine calcium/phosphorous (Ca/P) ratios in undecalcified teeth, and the sulfur (S) content of dentin of decalcified teeth from normal patients and patients with familial hypophosphatemia, in an attempt to determine the effect of phosphorus deficiency. The results s...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Daley TD,Jarvis A,Wysocki GP,Kogon SL

    更新日期:1990-12-01 00:00:00

  • Quantitative assessment of experimental fracture repair by peripheral computed tomography.

    abstract::An experimental fracture model was used to assess bone mineral density at the fracture site by peripheral computed tomography and to compare the model with biomechanical, histological, and radiographic methods for the quantification of the fracture repair process. Transverse osteotomies in the mid-diaphysis of 28 tibi...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Augat P,Merk J,Genant HK,Claes L

    更新日期:1997-02-01 00:00:00

  • Parathyroid hormone as a calcium ionophore in bone cells: tests of specificity.

    abstract::Parathyroid Hormone (PTH) stimulates calcium entry into bone cells in vitro, and this effect has been proposed to mediate the actions of PTH on bone. Using cells harvested from infant rat calvaria by collagenase digestion, we evaluated the sensitivity and specificity of this phenomenon. Calcium uptake was temperature ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Marcus R,Orner FB

    更新日期:1980-01-01 00:00:00

  • Modulation of bone mass, strength, and turnover by a new benzamide compound, DU-6712, in adjuvant-induced arthritic rats.

    abstract::To assess the reductions in bone mass, strength, and turnover in adjuvant-induced arthritic rats and to examine the effect of a new benzamide compound, DU-6712, on these parameters, a 28-day dosing experiment was performed using 8-week-old female Lewis rats. Arthritis was induced by injecting the adjuvant into the hin...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Uchiyama Y,Takamori H,Miyama K,Takano E,Nakajima H,Sato T,Nakamura T

    更新日期:1998-06-01 00:00:00

  • Activity increase after extraction of alkaline phosphatase from human osteoblastic membranes by nonionic detergents: influence of age and sex.

    abstract::The solubilization of alkaline phosphatase (AP) from osteoblastic cell membranes obtained from human primary bone cell cultures was studied according to the age and sex of the donors (17 females, 11 males; age range: 2-77 years). Cell membranes were treated by non-ionic (n-octyl beta-D-glucopyranoside, OG), ionic or z...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Bourrat C,Radisson J,Chavassieux P,Azzar G,Roux B,Meunier PJ

    更新日期:2000-01-01 00:00:00

  • Characterization and identification of a human dentin phosphophoryn.

    abstract::The present study further characterizes an extract from immature, human tooth apicies from which an intact dentin phosphoprotein has been identified. Third molar apicies from developing roots were decalcified in 10% EDTA until Ca2+ was undetectable in the decalcifying solution. The crude extract was run on 7.5% SDS-PA...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Chang SR,Chiego D Jr,Clarkson BH

    更新日期:1996-09-01 00:00:00

  • Treatment of Paget's disease of bone with a combination of intranasal salmon calcitonin and oral calcium and thiazide.

    abstract::The purpose of this study was to establish the smallest dose of nasally administered salmon calcitonin (SCT) which, if given in conjunction with a previously published calcium/thiazide treatment, would be as effective as parenteral SCT in the treatment of Paget's disease of bone. Forty patients suffering from symptoma...

    journal_title:Calcified tissue international

    pub_type: 临床试验,杂志文章


    authors: Evans RA,Somers NM,Dunstan CR,Hills E,Evans M

    更新日期:1991-09-01 00:00:00

  • Stimulatory effect of zinc on deoxyribonucleic acid synthesis in bone growth of newborn rats: enhancement with zinc and insulin-like growth factor-I.

    abstract::The effect of zinc on in vitro deoxyribonucleic acid (DNA) synthesis activity in the femoral-diaphyseal and metaphyseal tissues of newborn rats was investigated to determine a role of zinc in bone growth. In vitro DNA synthesis was assayed in a reaction mixture containing the 100 g centrifugation supernatant, which in...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Ma ZJ,Yamaguchi M

    更新日期:2001-09-01 00:00:00

  • Maximum grip strength is not related to bone mineral density of the proximal femur in older adults.

    abstract::In the past decade there have been numerous publications reporting a significant and direct relationship between handgrip strength and bone mineral density (BMD) of the proximal femur in older adults. The present report challenges the appropriateness of the methods, and thus the conclusions used in these studies. Spec...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Foley KT,Owings TM,Pavol MJ,Grabiner MD

    更新日期:1999-04-01 00:00:00

  • Developing antler, a model for endocrine regulation of bone growth. Concentration gradient of T3, T4, and alkaline phosphatase in the antler, jugular, and the saphenous veins.

    abstract::Thyroxine (T4), triiodothyronine (T3), and alkaline phosphatase (AP) were assayed monthly in white-tailed deer plasma obtained from the antler (A), jugular (J), and the saphenous (S) veins during the period of antler growth and the period of mineralization. The concentrations of T3, T4, and AP in the J and S were not ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Bubenik GA,Sempere AJ,Hamr J

    更新日期:1987-07-01 00:00:00

  • Local delivery of recombinant osteoprotegerin enhances postorthodontic tooth stability.

    abstract::Relapse after orthodontic tooth movement is a significant problem in orthodontics. The purpose of this study was to examine the efficacy of the osteoclast inhibitor osteoprotegerin-Fc (OPG-Fc) for inhibiting postorthodontic relapse. Rat maxillary molars were moved mesially and allowed to relapse for 24 days. Low-dose ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Hudson JB,Hatch N,Hayami T,Shin JM,Stolina M,Kostenuik PJ,Kapila S

    更新日期:2012-04-01 00:00:00

  • Pregnancy and Lactation-Associated Osteoporosis: Bone Histomorphometric Analysis and Response to Treatment with Zoledronic Acid.

    abstract::Pregnancy and lactation-associated osteoporosis (PAO) is a rare condition with little known pathophysiology. Most cases are diagnosed in the third trimester of pregnancy or in the first weeks postpartum, particularly in first pregnancies. Vertebral fractures are most commonly observed and characterised by prolonged se...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Grizzo FM,da Silva Martins J,Pinheiro MM,Jorgetti V,Carvalho MD,Pelloso SM

    更新日期:2015-10-01 00:00:00

  • Effects of loading rate on strength of the proximal femur.

    abstract::Results from previous quasi-static mechanical tests indicate that femurs from elderly subjects fail in vitro at forces 50% below those available in a fall from standing height. However, bone is a rate-dependent material, and it is not known whether this imbalance is present at rates of loading which occur in a fall. B...

    journal_title:Calcified tissue international

    pub_type: 临床试验,杂志文章


    authors: Courtney AC,Wachtel EF,Myers ER,Hayes WC

    更新日期:1994-07-01 00:00:00

  • Effects of Combined Whole-Body Electromyostimulation and Protein Supplementation on Local and Overall Muscle/Fat Distribution in Older Men with Sarcopenic Obesity: The Randomized Controlled Franconia Sarcopenic Obesity (FranSO) Study.

    abstract::The primary aim of the project was to determine the combined effect of whole-body electromyostimulation (WB-EMS) and protein supplements on local and overall muscle/fat distribution in older man with sarcopenic obesity (SO). Community-dwelling (cdw) men ≥ 70 years with SO were randomly allocated to a WB-EMS and protei...

    journal_title:Calcified tissue international

    pub_type: 杂志文章,随机对照试验


    authors: Kemmler W,Grimm A,Bebenek M,Kohl M,von Stengel S

    更新日期:2018-09-01 00:00:00

  • Regional measurement of bone calcium accretion rate and exchangeable pool with a whole-body counter: method and studies in subjects without bone disease.

    abstract::A whole-body counter was used to measure the retention and distribution of radioactivity along the longitudinal axis of the body during 10 days after the intravenous injection of 50 microCi of 47Ca. These data and the simultaneous measurements of the serum specific activity allowed us to calculate, by the Bauer-Carlss...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Bergmann P,Paternot T,Schoutens A

    更新日期:1983-01-01 00:00:00

  • Involvement of insulin-like growth factor I in development of ossification of the posterior longitudinal ligament of the spine.

    abstract::In order to investigate the pathogenesis of ossification of the posterior longitudinal ligament (OPLL) of the spine, we examined the distribution of insulin-like growth factor I (IGF-I) in the posterior longitudinal ligaments of OPLL patients, and analyzed the effects of IGF-I on the cultured spinal ligament cells. Fo...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Goto K,Yamazaki M,Tagawa M,Goto S,Kon T,Moriya H,Fujimura S

    更新日期:1998-02-01 00:00:00

  • Serum levels of bone Gla protein (osteocalcin, BGP) and carboxyterminal propeptide of type I procollagen (PICP) in acromegaly: effects of long-term octreotide treatment.

    abstract::We measured serum concentrations of bone Gla-protein (osteocalcin, BGP) and carboxyterminal propeptide of type I procollagen (PICP) in 14 patients with active acromegaly. Blood was collected at 0800 for measurement of bone Gla-protein (BGP), carboxyterminal propeptide of type I procollagen (PICP), and insulin-like gro...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Terzolo M,Piovesan A,Osella G,Pia A,Reimondo G,Pozzi C,Raucci C,Torta M,Paccotti P,Angeli A

    更新日期:1993-03-01 00:00:00

  • A controlled study of the effects of alendronate in a growing mouse model of osteogenesis imperfecta.

    abstract::Recent studies have reported that bisphosphonates reduce fracture incidence and improve bone density in children with osteogenesis imperfecta (OI). However, questions still persist concerning the effect of these drugs on bone properties such as ultrastructure and quality, particularly in the growing patient. To addres...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Camacho NP,Raggio CL,Doty SB,Root L,Zraick V,Ilg WA,Toledano TR,Boskey AL

    更新日期:2001-08-01 00:00:00

  • Epidemiology of fractures of the proximal femur associated with osteoporosis in Barcelona, Spain.

    abstract::We studied the epidemiology of osteoporotic fractures of the proximal femur (cervical and trochanteric fractures) in residents of Barcelona, aged 45 years or over, for a 1-year period. Fractures resulting from metastases were not included. During the period of observation, a total of 1,358 patients with hip fractures ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Diez A,Puig J,Martinez MT,Diez JL,Aubia J,Vivancos J

    更新日期:1989-06-01 00:00:00

  • TGF-beta/extracellular matrix interactions in dentin matrix: a role in regulating sequestration and protection of bioactivity.

    abstract::TGF-beta isoforms sequestrated in dentin matrix potentially provide a reservoir of bioactive molecules that may influence cell behavior in the dentin-pulp complex following tissue injury. The association of these growth factors with dentin matrix and the influence of such associations on the bioactivity of growth fact...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Baker SM,Sugars RV,Wendel M,Smith AJ,Waddington RJ,Cooper PR,Sloan AJ

    更新日期:2009-07-01 00:00:00

  • Muscle Function in Osteogenesis Imperfecta Type IV.

    abstract::Results of previous studies suggest that children and adolescents with osteogenesis imperfecta (OI) type IV have muscle force deficits. However, muscle function remains to be objectively quantified in this population. This study aimed to assess upper and lower extremity muscle function in patients with OI type IV. It ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Veilleux LN,Darsaklis VB,Montpetit K,Glorieux FH,Rauch F

    更新日期:2017-10-01 00:00:00

  • The small bovine amelogenin LRAP fails to rescue the amelogenin null phenotype.

    abstract::Amelogenins are the most abundant secreted proteins in developing dental enamel. These evolutionarily-conserved proteins have important roles in enamel mineral formation, as mutations within the amelogenin gene coding region lead to defects in enamel thickness or mineral structure. Because of extensive alternative spl...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Chen E,Yuan ZA,Wright JT,Hong SP,Li Y,Collier PM,Hall B,D'Angelo M,Decker S,Piddington R,Abrams WR,Kulkarni AB,Gibson CW

    更新日期:2003-11-01 00:00:00

  • Alkaline phosphatase in osteoblasts is down-regulated by pulsatile fluid flow.

    abstract::It is our hypothesis that interstitial fluid flow plays a role in the bone remodeling response to mechanical loading. The fluid flow-induced expression of three proteins (collagen, osteopontin, and alkaline phosphatase) involved in bone remodeling was investigated. Rat calvarial osteoblasts subjected to pulsatile flui...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Hillsley MV,Frangos JA

    更新日期:1997-01-01 00:00:00

  • In Vitro andIn Vivo induction of bone formation using a recombinant adenoviral vector carrying the human BMP-2 gene.

    abstract::It has been well established that bone morphogenetic protein-2 (BMP-2) can induce bone formation bothin vivo andin vitro, although high concentrations (up to milligrams) of BMP-2 have been required to achieve this effectin vivo. Further, clinical applications are usually limited to a single dose at the time of implant...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Cheng SL,Lou J,Wright NM,Lai CF,Avioli LV,Riew KD

    更新日期:2001-02-01 00:00:00

  • Crystal dissolution of biological and ceramic apatites.

    abstract::High resolution transmission electron microscopy (Hr TEM) studies on biological and synthetic calcium phosphate have provided information on the dissolution process at the crystal level. The purpose of this study was to investigate the dissolution of ceramic hydroxyapatite (HA) after implantation using Hr TEM. Recover...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Daculsi G,LeGeros RZ,Mitre D

    更新日期:1989-08-01 00:00:00

  • A new culture method assuring the three-dimensional development of the mouse embryonic molar tooth in vitro.

    abstract::Mandibular first molars from 17-day-old mouse embryos were cultivated in vitro for 10 days using a new organ culture method. This method consisted of using a small glass dish and an agar chamber to slowly float the molars to the gas-medium interface where they were maintained by the surface tension of the medium. The ...

    journal_title:Calcified tissue international

    pub_type: 杂志文章


    authors: Sakakura Y

    更新日期:1986-10-01 00:00:00