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Elastin<br />

Clone BA-4<br />

0.5 mL lyophilized NCL-ELASTIN P (Enzyme)<br />

Elastin is a polymeric protein found in connective tissue which imparts the<br />

property of elasticity to vertebrate elastic tissue. It is synthesized and<br />

secreted as a soluble, single-chain protein (tropoelastin) which undergoes a<br />

number of post-ribosomal modifications prior to its organization into an<br />

elastic fiber in the extracellular space. Once secreted, tropoelastin<br />

molecules are joined covalently via chemical modification and cross-linking<br />

of specific lysyl residues to form the mature insoluble elastin.<br />

Ultrastructurally, it is predominantly an amorphous material which may<br />

change its morphology with ageing and different disease states. The<br />

abnormal accumulation of elastic tissue in blood vessels is found in<br />

atherosclerosis and hypertension. Genetic defects in the elastin molecule<br />

are reported to lead to inherited diseases such as Marfan's syndrome,<br />

pseudoxanthoma elasticum and the Bushke-Ollendorf syndrome.<br />

Human aorta: immunohistochemical staining for elastin using NCL-ELASTIN. Note extracellular<br />

staining within the arterial wall. Paraffin section.<br />

Emerin<br />

Clone 4G5<br />

1 mL, 0.1 mL lyophilized NCL-EMERIN F P (HIER) W<br />

Emery-Dreifuss muscular dystrophy (EDMD) is a late onset X-linked<br />

recessive disorder characterized by slowly progressing contractures,<br />

wasting of skeletal muscle and cardiomyopathy usually presented as heart<br />

block. Contractures are seen in the elbows, Achilles tendons and<br />

postcervical muscles with humero-peroneal distribution early in the course<br />

of the disease. The STA gene, at Xq28 locus, encodes a serine-rich 34 kD<br />

protein, emerin, which is ubiquitous in tissues and is found in highest<br />

concentration in skeletal and cardiac muscle. Emerin is localized in the<br />

nuclear membrane of normal muscle cells and its deficiency plays a crucial<br />

part in the pathology of EDMD.<br />

Product Specific Information<br />

NCL-EMERIN is of use in the detection of the normal STA gene product.<br />

Human skeletal muscle: immunohistochemical staining for emerin using NCL-EMERIN.<br />

Note perinuclear staining of all cell nuclei. Paraffin section.<br />

Endoglin (CD105)<br />

Clone 4G11<br />

1 mL, 0.1 mL lyophilized NCL-CD105 P (HIER)<br />

CD105, also known as Endoglin, is an endothelial homodimeric membrane<br />

glycoprotein containing the peptide sequence RGD which is a recognition<br />

motif for adhesion receptors of the integrin family. It has been proposed that<br />

endoglin is a TGF-beta receptor. CD105 antigen is reported to be expressed<br />

on endothelial cells of capillaries, arterioles and venules in a variety of<br />

tissues and at low levels on acute lymphoblastic and myelocytic leukemia<br />

cells. ENDOGLIN expression may be of interest in the study of monocyte<br />

differentiation into macrophages, studies of cellular adhesion of circulating<br />

blood cells and in the lysis of CD105 positive cells in the presence of<br />

complement.<br />

Normal human ovary: immunohistochemical staining for endoglin using NCL-CD105. Note<br />

membrane staining of cells in both the theca interna and theca externa. Paraffin section.<br />

Endothelial Cell Marker (CD34)<br />

Clone QBEnd/10<br />

1 mL, 0.1 mL lyophilized NCL-END F P (Enzyme) C<br />

1 mL liquid NCL-L-END F P (Enzyme) C<br />

7 mL ready-to-use RTU-END F P (Enzyme)<br />

7 mL Bond ready-to-use PA0212 P (HIER)<br />

Refer to page 77 for further information about CD34.<br />

Endothelin-1 Receptor (ETA)<br />

Clone RJT24<br />

1 mL, 0.1 mL liquid NCL-L-ETA P (HIER)<br />

Endothelins (ET) are potent vasoconstrictive peptides originally isolated<br />

from vascular endothelial cells. Their biological effects are mediated<br />

through two different receptors, endothelin-1-selective endothelin receptor<br />

(ETA) and the non-selective receptor sub-type (ETB). Analysis of mRNA by<br />

Northern blotting reported high levels in aorta, lung, atrium, colon and<br />

placenta with moderate levels in the cerebral cortex, cerebellum, ventricle,<br />

kidney, adrenal glands and duodenum, whereas liver was negative. Studies<br />

using immunohistochemistry have reported the presence of ETA in medial<br />

smooth muscle of arteries, full-term placenta, normal and diseased gall<br />

bladder and ovarian luteinized granulosa cells. ETA has also been reported<br />

to have a role in gastric ulcer healing and in the development of neural-crest<br />

derived cells.<br />

F Frozen I Immunofluorescence E Electron microscopy<br />

P Paraffin C Flow cytometry O Other applications<br />

W Western blotting<br />

/ 103<br />

Primary Antibodies

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