Creutzfeldt-Jakob 14-3-3 Protein

Last Modified: 6/27/2019 12:57:55 PM

Medical Necessity Documentation:  
Client Notes:  
Patient Preparation:  
Specimen Requirements: 1.0 mL CSF in a Sterile Plastic Vial
Collection Instructions: 1. Obtain aliquot from second collection vial. 
2. Hemolyzed specimens will give false-positive results. Specimens should be centrifuged to remove any red cells before shipping. The test will be canceled if there is any level of hemolysis present. 
3. Immediately place aliquot on ice. 

Additional Information: 
Specimens that have not been kept refrigerated, or which have been tested for other analytes previously, may give a false-positive result. 
Separate specimens should be submitted when multiple tests are ordered. This will reduce the risk of test cancellation due to stability problems.
Minimum Volume: 0.6 mL CSF on ice
Transport & Storage: Temperature/Stability: 7 days Refrigerated 
90 days Frozen
Rejection Criteria: Hemolysis
Reference Range:

Normal: < or =2.0 ng/mL

Elevated: >2.0 ng/mL

Critical Ranges:  
Test Comments:  
Methodology: Immunochemiluminometric Assay (ICMA)
Clinical Significance:

The 14-3-3 proteins are a group of highly conserved proteins composed of several isoforms that are involved in the regulation of protein phosphorylation and mitogen-activated protein kinase pathways. They exist in vivo as dimers of the various isoforms with apparent molecular mass of 30 kDa on sodium dodecyl sulfate polyacrylamide gel electrophoresis and 60 kDa on gel chromatography. Sequence homology among the various isoforms ranges from 22% to100%. The beta, gamma, and theta isoforms are found in tissues of the nervous system.

Detectable 14-3-3 protein in the cerebrospinal fluid (CSF) is indicative of substantial, relatively rapid neuronal destruction. Increased CSF concentrations of 14-3-3 proteins have been described in patients with various forms of Creutzfeldt-Jakob disease (CJD), some other rapidly progressive dementias, and a large range of other vascular, inflammatory, neoplastic, and metabolic central nervous system (CNS) disorders (see Cautions), which can be associated with significant and rapid neuronal destruction.

The main clinical use of 14-3-3 measurements is in the differential diagnosis of dementia, in particular to distinguish CJD and its variants from other dementias. The most common forms of dementia (progressive multi-infarct dementia and Alzheimer disease) are uncommonly associated with elevated CSF levels of 14-3-3, presumably because of their slow pace of progression.

CJD is an incurable neurodegenerative disease caused by accumulation of self-catalytically malfolded endogenous prion proteins in the CNS. Its cause is most commonly sporadic, but it can be inherited (mutations that predispose to malfolding) or acquired (iatrogenic transmission by infected human tissues or tissue extracts or surgical procedures, or by ingestion of some animal products that contain malfolded prion proteins).

The diagnosis of CJD is highly complex and involves clinical history and neurologic examination, electroencephalographs (EEG), magnetic resonance imaging (MRI), and exclusion of other possible causes of dementia, in addition to CSF examination. Several, slightly different scoring systems are in use to integrate these parameters into a final diagnosis of possible, probable, or definite CJD. The most widely accepted of these scoring systems is the WHO set of diagnostic criteria for sporadic CJD from 1998 (see Interpretation). Supporting, in conjunction with other tests, a diagnosis of Creutzfeldt-Jakob disease in patients with rapidly progressive dementia when other neurodegenerative conditions have been excluded

Custom Panel: No


Turn Around Time: 2 to 6 days
Days Performed: Monday, Thursday
Sites Performed: Mayo Laboratory
PHL Test Code: MSOT
Send Out Test Code: P1433
Alternate Test Names: 14-3-3 Protein, CSF; 14-3-3 Protein, Spinal Fluid; CJD; Cretuzfeld-Jakob; Protein 14-3-3
Included Tests:  
CPT Coding: 83520

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