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Assays to quantify total huntingtin (tHTT) and mutant huntingtin (mHTT) protein in vitro and in vivo

IRBM has pioneered sensitive and selective assays to measure Huntingtin (HTT) protein. Mutations in the huntingtin gene and resulting protein causes Huntington’s disease (HD). IRBM’s HTT protein assays can detect disease-causing polyglutamine repeats by measuring proteins in a polyglutamine-independent (both mutant expanded and non-expanded proteins), or a polyglutamine length-dependant manner.

We have a comprehensive suite of mHTT/HTT assays that use state-of-the-art technologies to measure protein and mRNA. We have the capability to measure HTT and mHTT expression in vitro, in animal models and in human samples such as CSF, saliva, blood and brain tissue, with a sensitivity of <5fM.

IN VITRO SAMPLES:

Cell lines:

  • Various Lab cell lines including genetically modified
  • Lymphoblastoids for routine HTT lowering testing (weekly)

Primary cells:

  • Striatal and cortical neurons and astrocytes for mice and rats

IPSC:

  • NPCs and terminally differentiated
  • Fully characterized

ANIMAL SAMPLES:

Mouse:

  • R6/2, Q175, BACHD, humanized models (Hu)
  • Tissues:
    • Forebrain
    • Striatum
    • Cortex
    • Cerebellum
    • Olfactory bulb
    • Spinal cord
    • Quadriceps
    • Liver
    • Spleen
    • Blood (and compartments)
    • Heart
    • Lung
    • Kidney
  • BACHD rat (brain, CSF and peripheral tissues)
  • Minipig (brain and CSF)
  • Sheep (brain and CSF)
  • NHP wt and transgenic (brain, blood and CSF)

HUMAN SAMPLES:

  • CSF
  • Blood
    • Whole blood
    • Plasma
    • PBMCs
    • Platelets
    • Granulocytes
  • Post-mortem brain
  • Saliva

Non-GLP clinical studies ongoing at IRBM

ASSAYS:

Proteins

  • mHTT
  • PolyQ-independent HTT
  • Aggregated HTT
  • NFL
  • HbA
  • Total proteins

mRNA:

  • Standard real time
  • Experience with splicing acting compounds
  • Skilled in mRNA and protein extraction from the same sample

Depending on the number, volume and type of samples, we’ll use either our Single Molecule Counting (SMC), or our electrochemiluminescence Meso Scale Discovery (MSD) detection system.  Our bioanalytical team has extensive experience in handling all types of biomarkers, including small molecules, oligos, peptides, and proteins, from sample preparation (PPT, SPE, LLE, IP) to sample analysis (qPCR, LBA, LC-MS/MS, LC-HRMS).

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