Primary ciliary dyskinesia encompasses a heterogenous group of inherited condition characterized by clinical, functional, and ultrastructural features. The transmission electronmicroscopic findings of nasal cilia in a 14-year old girl with primary ciliary dyskinesia were studied. The ultrastructure of axonemes showed normal outer membrane, dynein arms, microtubules, and nexin links but partial lack of radial spokes. The nature of the defective spoke is not clear and further studies will be necessary to determine how the radial spokes and central sheath interact and coordinate ciliary movement.
Adolescent ; Arm ; Axoneme ; Cilia ; Dyneins ; Female ; Humans ; Kartagener Syndrome* ; Membranes ; Microtubules

Ciliogenesis was investigated in the tracheal epithelium of human fetus at mid trimester of gestation (15~22 weeks), and the substructure of basal body was studied with serial, cross sections. The ciliogenic cells were long columnar cells with an electron -lucent cytoplasm, and contained rich free ribosomes and smooth endoplasmic reticulum. Apical cytoplasm of these cells contained various structures related to ciliogenesis including fibrous granules, procentrioles, centrioles and basal bodies. Basal bodies were located near apical plasma membrane and had basal foot and striated rootlets. In cross section, alar sheets appeared at transitional area between distal portion of basal body and axoneme, and basal foot at distal portion of basal body. Alar sheets arouse from each peripheral triplets of basal body and projected radially clockwise in apex to base view. Basal foot was a cone shaped structure with cross striation which base attached to two or three of the peripheral triplet sets and apex converged to basal foot cap. Three dimentional reconstruction by serial cross section of the basal body showed a structural relationship of alar sheets and basal feet with basal body. By immunohistochemistry, alpha -tubulin label was seen in both basal and surface ciliated cells, and gamma-tubulin label was seen in the apical region of surface cilated cells. These results indicate that ciliogenesis of tracheal epithelium of human fetus is performed mainly by acentriolar ciliogenesis, and suggest the ciliogenesis and ciliary movement at mid trimester of gestation are active.
Axoneme ; Basal Bodies ; Cell Membrane ; Centrioles ; Cytoplasm ; Endoplasmic Reticulum, Smooth ; Epithelium* ; Fetus* ; Foot ; Humans* ; Immunohistochemistry ; Pregnancy ; Ribosomes ; Triplets ; Tubulin

Giardia lamblia is a protozoan that causes diarrheal diseases in humans. Cytoskeletal structures of Giardia trophozoites must be finely reorganized during cell division. To identify Giardia proteins which interact with microtubules (MTs), Giardia lysates were incubated with in vitro-polymerized MTs and then precipitated by ultracentifugation. A hypothetical protein (GL50803_8405) was identified in the precipitated fraction with polymerized MTs and was named GlMBP1 (G. lamblia microtubule-binding protein 1). Interaction of GlMBP1 with MTs was confirmed by MT binding assays using recombinant GlMBP1 (rGlMBP1). In vivo expression of GlMBP1 was shown by a real-time PCR and western blot analysis using anti-rGlMBP1 antibodies. Transgenic G. lamblia trophozoites were constructed by integrating a chimeric gene encoding hemagglutinin (HA)-tagged GlMBP1 into a Giardia chromosome. Immunofluorescence assays of this transgenic G. lamblia, using anti-HA antibodies, revealed that GlMBP1 mainly localized at the basal bodies, axonemes, and median bodies of G. lamblia trophozoites. This result indicates that GlMBP1 is a component of the G. lamblia cytoskeleton.
Antibodies ; Axoneme ; Basal Bodies ; Blotting, Western ; Cell Division ; Cytoskeleton ; Fluorescent Antibody Technique ; Giardia lamblia* ; Giardia* ; Hemagglutinins ; Humans ; Microtubules ; Polymers ; Real-Time Polymerase Chain Reaction ; Trophozoites

axoneme and manchette are crucial for sperm head and tail formation. This review (a) presents a concise, yet detailed structural overview of the microtubules, (b) analyses the role of microtubule structures in various male reproductive functions, and (c) presents the association of microtubular dysfunctions with male infertility. Considering the immense importance of microtubule structures in the formation and maintenance of physiological functions of sperm cells, this review serves as a scientific trigger in stimulating further male infertility research in this direction.]]>
Actin Cytoskeleton ; Axoneme ; Cytoskeleton ; Humans ; Infertility, Male ; Kartagener Syndrome ; Male ; Male ; Microtubule-Associated Proteins ; Microtubules ; Organelles ; Sperm Head ; Sperm Motility ; Spermatogenesis ; Spermatozoa ; Tail

Kartagener's syndrome, a congenital disease transmitted as an autosomal recessive illness with a prevalence of approximately 1:20,000 persons, is characterized by the triple association of situs inversus, bronchiectasis, and sinusitis. Affected persons have an incoordination of ciliary motility that leads to defective mucociliary transport, chronic bronchial infections. Kartagener's syndrome is a subset of the immotile cilia syndrome and therefore all patients with Kartagener's syndrome have immotile cilia with obvious ultrastructural defects in the ciliary axoneme. In the respiratory tract this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of the sinuses and bronchial trees that are characterized of the disease. The end-stage phenomenon in Kartagener's syndrome, respiratory or heart failure is a less common event and heart-lung transplantation is becoming an accepted therapy for patients with end-stage pulmonary disease in Kartagener's syndrome in many institutes. We report one case of Kartagener's syndrome in a 25-year-old young woman who was presented as respiratory and right heart failures, with review of literatures.
Academies and Institutes ; Adult ; Ataxia ; Axoneme ; Bronchiectasis ; Cilia ; Ciliary Motility Disorders ; Female ; Heart Failure ; Heart* ; Heart-Lung Transplantation ; Humans ; Kartagener Syndrome* ; Lung Diseases ; Mucociliary Clearance ; Mucus ; Prevalence ; Respiratory Insufficiency ; Respiratory System ; Sinusitis ; Situs Inversus ; Trees