Creutzfeldt-Jakob disease deaths and death rates by age group, sex, race, and region, United States, 1979–2006.^*

*<p>Deaths obtained from the multiple cause-of-death data for 1979–1998 are based on ICD-9 codes, and deaths beginning in 1999 are based on ICD-10 codes with available computerized literal death certificate data. Death information was also obtained from other surveillance mechanisms. Death rates expressed per 1,000,000.</p>†<p>One death is missing both race and region; and one death is missing region.</p

Creutzfeldt-Jakob disease deaths and death rates by age group, region and race, United States, 1979–2006.^*

*<p>Deaths obtained from the multiple cause-of-death data for 1979–1998 are based on ICD-9 codes, and deaths beginning in 1999 are based on ICD-10 codes with available computerized literal death certificate data. Death information was also obtained from other surveillance mechanisms. Death rates expressed per 1,000,000. One death missing is both race and region; and one death is missing region.</p>†<p>Death rates are age-adjusted.</p

Creutzfeldt-Jakob disease age-specific and age-adjusted death rates, United States, 1979–2006.

<p>Creutzfeldt-Jakob disease age-specific and age-adjusted death rates, United States, 1979–2006.</p

Creutzfeldt-Jakob disease deaths and death rates by age group, United States, 1979–2006.

<p>Creutzfeldt-Jakob disease deaths and death rates by age group, United States, 1979–2006.</p

Creutzfeldt-Jakob disease deaths and age-adjusted death rates, United States, 1979–2006.

<p>Creutzfeldt-Jakob disease deaths and age-adjusted death rates, United States, 1979–2006.</p

Peripheral tissues shown to contain PrP^res in vCJD¹.

1<p>Data obtained from immunoblotting and immunohistochemistry studies. Brown P., unpublished data; WHO Expert Committee Meeting, Baden, Austria, 18 May 2007 (updated through Jan 2009).</p

Characteristics of PK-sensitive PrP.

<p>Immunoblot analysis of total homogenate from brain, pituitary gland and uterus are shown. The samples, with or without previous PK treatment, were deglycosylated with PNGase F. Membranes were probed with the mAbs 3F4 and 2301 as indicated. <b>A:</b> The brain has relatively large amounts of full-length isoform and PrP^res C2 fragment but the N-terminus truncated PrP^C fragment (C1) is poorly represented. In addition, brain preparation shows a previously unreported PK-sensitive fragment with molecular weight of 25 kDa (arrow), detectable only in deglycosylated samples, of undetermined origin. <b>B:</b> The C1 fragment is relatively better represented in the pituitary gland <b>C:</b> while it is overly abundant in the uterus.</p

Creutzfeldt-Jakob disease death rates and deaths, United States, 1979–2006.^*

*<p>Deaths obtained from the multiple cause-of-death data for 1979–1998 are based on ICD-9 codes and those beginning in 1999 are based on ICD-10 codes with available computerized literal death certificate data. Death information was also obtained from other surveillance mechanisms. One death is missing both race and region; and one death is missing region.</p>†<p>Death rates expressed per 1,000,000 persons in corresponding group. Death rates and risk ratios are age-adjusted for total, sex, race, and region. The 95% confidence intervals (CIs) are given.</p>‡<p>p<0.001 for comparison of death rate to that of the referent group.</p

Detection of PrP^res in non-nervous tissues.

<p><b>A:</b> PrP^res from dura mater and frontal cortex (1∶24 dilution) from the present case is compared to PrP^res of the frontal cortex from sCJDMM1 (type 1) and sCJDMM2 (type 2). <b>B:</b> Two film exposures of immunoblots from non-nervous tissues compared with that of the frontal cortex (1∶10 dilution). Pituitary gland, adrenal gland and uterus are clearly positive while the bands in the remaining preparations are considered to be non specific. <b>C:</b> PrP^res from skin (double TH loading, equivalent to 4 mg of wet tissue) is barely detectable in TH (Lane 3) compared with frontal cortex TH, which is diluted 1∶4 (lane 1) or 1∶130 (lane 2). Skin PrP^res is better detectable along with the kidney PrP^res after sodium phosphotungstate (NaPTA) precipitation of PrP^res (Lanes 4 and 5), especially after long exposure (Lanes 4L and 5L) (kidney TH loaded in double amount; probed with mAb 3F4). <b>D:</b> PrP^res from mesenteric lymph nodes and other visceral organs recovered following NaPTA precipitation and compared with TH from the frontal cortex (1∶120 dilution) and sCJDMM1 following two film exposures. All organs but ascending colon are positive. Of note, unglycosylated isoform is underrepresented in all NaPTA precipitated samples as compared with that of the TH preparations (see panel A, lanes 3 and 4 and panel B). <b>A–D:</b> Membranes were probed with the mAb 3F4.</p

Synopsis of PrP^res analyses in the brain and other tissues.

1<p>In <b><i>bold italic</i></b> organs where PrP^res had not been detected previously.</p>2<p>TH: PrP^res searched in tissue homogenate.</p>3<p>NaPTA: PrP^res searched following enrichment with sodium phosphotungstate precipitation.</p>4<p>Amount of PrP^res expressed as percentage of PrP^res present in the frontal cortex. Glyc. ratio: glycoform ratio expressed as percentage of the sum of the three isoforms and representing diglycosylated:monoglycosylated:unglycosylated forms. Data listed for tissue positive in both TH and NaPTA preparations were obtained from TH.</p>5<p>PrP^res was previously reported in colon with no specification of the segment examined.</p