Soft tissue anomalies in a patient with congenital tibial aplasia and talo-calcaneal synchrondrosis

We have carried out the third in a series of anatomical dissections of amputated congenitally deformed human limbs in an attempt to determine the etiological relationship between the bony and soft tissue anomalies. This specimen consisted of a limb with congenital tibial aplasia and an adducted foot with five toes. The arterial and nerve patterns were reminiscent of those seen previously by us. The arteries were characterized by absence of the anterior tibial artery and an incomplete plantar arch. The superficial peroneal nerve terminated at the ankle. Extra branches from the sural, deep peroneal, and medial plantar nerves supplied branches to the dorsum of the foot. There were two extra muscles in the leg. The remaining muscles were normal, with the exception that most muscles normally inserting on the plantar surface of the foot inserted instead into a common tendon sheet. The tibia was replaced by a tendinous band, and the talus and calcaneus were united by a complete synchondrosis. The contralateral limb clinically appeared to have a clubfoot. The combination of an absent anterior tibial artery and an incomplete plantar arch is consistent with our theory that a reduced number of vessels puts the embryonic limb at increased risk of congenital defects due to the reduction in the number of collateral blood routes. Some event, such as extravasation of blood or embolization, may concurrently or subsequently compromise blood flow in the remaining vessels. We have previously observed abnormal arterial patterns similar to that described above in limbs having absence of the tibia and in other patients having clubfeet. The presence of abnormal arterial patterns in a limb with absence of the tibia and a contralateral limb with clubfoot suggests that absent tibia and clubfoot may be etiologically related.     

Effects of temperature and moisture during incubation on carcass composition of hatchling snapping turtles (Chelydra serpentina)

Summary Flexible-shelled eggs of common snapping turtles (Chelydra serpentina) were incubated on each of two substrates (vermiculite, sand) at each of three temperatures (26.0°C, 28.5°C, 31.0°C) and three moisture regimes (wet, intermediate, dry). Embryos developing in cool, wet environments mobilized the largest amounts of protein from their yolk and attained the largest size before hatching, whereas turtles developing in warm, dry environments mobilized the smallest quantities of protein and were the smallest in body size at hatching. Embryos on wet substrates mobilized more lipid from their yolk than did embryos on dry media, but ambient temperature had no demonstrable influence on patterns of lipid mobilization. The total reserve of neutral lipid available in residual yolk plus carcass to sustain neonates in the interval prior to the beginning of feeding was largest in hatchlings from dry environments and smallest in animals from wet environments, but was unaffected by temperature during incubation. Hydration of tissues in hatchlings was higher when incubation was in cool, moist conditions than when incubation was in warm, dry settings, thereby indicating that some of the effects of moisture and temperature on mobilization of nutrients by embryos may be mediated by differences in intracellular water. Patterns of response to temperature and moisture recorded for turtles emerging from eggs on sand were similar to those recorded for hatchlings on vermiculite, so no important conclusion would have been affected by incubating eggs on one medium instead of the other.     

Lipoprotein metabolism in hepatic lipase deficiency: studies on the turnover of apolipoprotein B and on the effect of hepatic lipase on high density lipoprotein

Hepatic lipase deficiency produces significant distortion in the plasma lipoprotein profile. Particles with reduced electrophoretic mobility appear in very low density lipoprotein (VLDL). Intermediate density lipoprotein (IDL) increases markedly in the circulation and plasma low density lipoprotein (LDL) levels fall. At the same time there is a mass redistribution within the high density lipoprotein (HDL) spectrum leading to dominance in the less dense HDL2 subfraction. The present study examines apolipoprotein B turnover in a patient with hepatic lipase deficiency. The metabolism of large and small very low density lipoproteins was determined in four control subjects and compared to the pattern seen in the patient. Absence of the enzyme did not affect the rate at which large very low density lipoproteins were converted to smaller particles within this density interval (i.e., of VLDL). However, subsequent transfer of small very low density lipoproteins to intermediate density particles was retarded by 50%, explaining the abnormal accumulation of VLDL in the patient's plasma. Despite this, intermediate density particles accumulated to a level 2.4-times normal because their subsequent conversion to low density lipoprotein has been almost totally inhibited. Consequently, the plasma concentration of low density lipoprotein was only 10% of normal. On the basis of these observations, hepatic lipase appears to be essential for the conversion of small very low density and intermediate density particles to low density lipoproteins. The pathways of direct plasma catabolism of these species were not affected by the enzyme defect. In vitro studies were performed by adding purified hepatic lipase to the patient's plasma.(ABSTRACT TRUNCATED AT 250 WORDS)     

Site-directed labeling of a monoclonal antibody: targeting to a disulfide bond

We have designed and synthesized crabescein, the first member of a class of fluorescent labels that add across disulfide bonds. Crabescein is a fluorescein derivative that reports the rotational correlation time of the immunoglobulin G (IgG) segment to which it is covalently bound. Chemical analysis of the IgG labeled with crabescein indicates that the fluorophore is inserted into the third disulfide bond (cysteine-229 of mouse IgG2a) in the hinge region. The rotational correlation time of this labeled macromolecule was measured as a single exponential with a decay constant of 26.8 ns. This is in contrast to the double exponential with decay constants of 14.3 and 0.2 ns for the same IgG when labeled with fluorescein via a conventional labeling reagent in which the probe is bound to the macromolecule by one-point attachments. Thus, crabescein is the prototype of a class of fluorescent and phosphorescent probes that, by virtue of their two-point attachments to proteins, faithfully report on the dynamics of the segment of macromolecule to which they are covalently bound.     

A new species ofScymnodalatias from the southern oceans,and comments on other squaliform sharks

Scymnodalatias albicauda sp. nov. is described from two specimens taken at high latitudes (45°S and 49°S). It is distinguished fromS. sherwoodi, only known species of the genus, by having white markings on the caudal fin, the second dorsal posterior tip almost reaching the upper caudal fin, shorter snout and head, smaller eye and larger fins. Relationships ofScymnodalatias to the generaScymnodon, Centroscymnus, andZameus are discussed, based chiefly on dermal denticle structure.Scymnodalatias andZameus uniquely share transverse ridges on their dermal denticles, and on this character they are treated as sister-groups. Comments on the above genera,Z. squamulosus and some species ofScymnodon are made to clarify their systematic status. As a result, it is proposed thatScymnodon includesichiharai, macracanthus, plunketi, andringens, thatCentroscymnus includescoelolepis, crepidater, crypt acanthus, andowstonii, and thatZameus includessquamulosus.     

Effects of sulfhydryl and other reagents on the diffusional permeability of dog erythrocytes to small solutes

The effects of p-chloromercuriphenylsulfonic acid (PCMBS), 5,5'-dithiobis (2-nitrobenzoic acid) (DTNB), phloretin and thiourea on the diffusional permeability of dog erythrocytes to tritiated water and to small 14C-labeled lipophilic and hydrophilic solutes were measured at 37 degrees C by means of the linear diffusion technique. Permeability to 3HHO was significantly decreased by PCMBS but was not affected by the other reagents. The permeability to the small hydrophilic solutes acetamide and urea was decreased by phloretin and thiourea but only the permeability to acetamide was reduced to a statistically significant extent by PCMBS. The permeability to the lipophilic solutes methanol, ethanol and antipyrine was not affected by any of these agents. We interpret these results as an indication that the small lipophilic solutes probably move through lipid areas, that the small hydrophilic solutes probably move through protein associated areas in the erythrocyte membrane and that pathways for the small hydrophilic solutes are distinct from those for water. While the pathways for water may be associated with membrane protein they do not appear to be associated specifically with band 3 protein as has been suggested for human erythrocytes. Diffusional water movement through the dog erythrocyte occurs by two distinct pathways.