Parkin

BrainCSF
NSTM SMCxPro0.41 pg/mg0.06 pg/mL with 1 mL CSF
DMPK LC-MS/MS32.5 pg/mg protein
DMPK SIMOA0.137-0.412 pg/mL (question: 0.244 pg/mL 을 위해선 NSTM 은 250 ul CSF 필요한데 어떻게 dmpk 는 50? 이면 되는지?)

pS65-Ub assays

Detection Ab (for fluorescent label)Capture Ab (for magnetic bead)Recombinant Ps65Ub
TakedaSMCxProAnti-Ub antibody (detection): P4D1 (Cell Signaling Technology, Cat# 3936, mouse monoclonal antibody)Anti-pS65Ub antibody: E2J6T (Cell Signaling Technology, Cat# 62802, rabbit monoclonal antibody)
  • K48 Phospho-tetra-ubiquitin Chains (pS65-poly Ub, Boston Biochem, Cat#: UC-250)
  • Phosphor-ubiquitin monomer (pS65-mono Ub, Boston Biochem, Cat#: U-102)
  • K48 tetra-ubiquitin (poly-Ub, Cat# SBB-UP0070, South Bay Bio)
MitokininSMCxProUbiquitin:
Ub P4D1 (many sources)
Phospho-ub:
i) E2J6T clone (Cell Signaling)
K48 pS65-Ub (standard-Biotechne Catalog # UC-250)
Wolfdieter Springerhttps://www.michaeljfox.org/grant/ps65-ub-biomarker-parkinsons-disease
(Watzlawik, 2020 #1760)
Ubiquitin (including P4D1)Phospho-ub (including E2J6T)
Shalini Padmanabhan
spadmanabhan@michaeljfox.org

Different Ubiquitin linkage

Ubiquitination occurs through a linkage between the C-terminus of ubiquitin and the e-amino group of a lysine residue on the target substrate.

Ubiquitin itself has seven Lys residues (K6, K11, K27, K29, K33, K48, and K63).

Ubiquitin to form polymers through various lysine

IVC

TopicTypical CSF volume per drawAction
Rat100 uLVery low feasibility, mitigation? 5 rats pooled to make 500 ul (ie minimum), For 100 ul, LLOQ is 0.6 pg/mL, so then window is only 2.2x.
NHP1 mL

Parkin activity assay?

Ps65-ubParkin protein assayParkin activity assay (this should mean E3 ligase activity assay)
prosReflect the feedforward process to form polyubiquitinated chain which is a concerted effect of PINK1 and parkin, enabling lysosomal mitophagy.

In the physiologic mitophagy process, the elongation of ps65-ub chain is the function of Parkin. (PINK1 is responsible for phosphorylation of ub)
  • May be a direct measure of Parkin activity
  • Can reflect the total capability of Parkin (ie endogenous + exogenous), so maybe more suitable for PK-PD relationship assessment
ConsMay not recognize certain truncated parkin depending on mutation (then only reflecting exogenous parkin when the endogenous parkin still has enzyme activity)The assay is somewhat arbitrary because PINK1, ubiquitin E1, and E2 conjugating enzyme need to be added to enable the Parkin reaction.

Eg. https://southbaybio.com/products/parkin-tr-fret-ubiquitin-kit.html
(this is the one Takeshi considered for in vivo studies assay) TR-FRET detection of ubiquitin chain formation onto Parkin (js: this is not specific to phospho-ub!)
questionWhat is the active site in the parkin protein? it is catalytic cysteine residue (Cys431) on exon12.Feasible in CSF? Probably yes

TMQB

Oct 26 27 2021 Parkin GT TMQB draft.pptx

Objectives of TMQB meeting

Provide cross-functional translational leadership input to project teams on pre-CS TMPs

Align cross-functional translational input to DDU Strategy Investment Board (SIB) wherever possible

Proposed Schedule of PARKIN GT TMQB preparation schedule

Oct 12, 2021: 1st draft to Jaewon

Oct 12 - Oct 23, 2021: review and revisit (Team)

Oct 24, 2021: Meeting materials submission

TMQB meeting: Wednesday, October 27, 2021 8:00 AM-9:55 AM (Japan time)

TopicContentsResponsible authorPresenter at TMQB
Translational Probability of Success (TPoS)Slide template providedJaewonJaewon
Executive SummaryJaewonJaewon
Target validationHuman genetic/post-mortem data
Animal models of disease mechs
TakeshiTakeshi
Current status of asset generationin vitro and in vivo pharmacology
project timeline
TakeshiTakeshi
SafetyTSR
potential safety biomarkers, and necessary de-risking
Oak-sanOak-san
Quantitative Pharmacology TargetsPK-PD relationships
prediction of human response
Nishihara-sanNishihara-san
Clinical biomarker feasibilityBMx development timeline slide
Biomarker Usage Map (“Danhoff Slide”)
JaewonJaewon
TopicPresenter at TMQBtime (min)
Executive SummaryJaewon2
Target validationTakeshi15
Current status of asset generationTakeshi
SafetyOak-san10
QP/PDNishihara-san10
BiomarkersJaewon15
Translational Probability of Success (TPoS)Jaewon8
Total60

TPP/TCP

Working document of TCP slides

Working document Parkin GT Target Candidate Profile (TCP).pptx

Biodistribution: my rationale

  • Gliosis widespread in the brain
    • Lifelong progressing disorder, cortical involvement is matter of time.
    • Even when limited in motor:
      • When diagnosed, 60% of DA neuronal loss in SN, ie PD is a circuit disorder, before then, neuronal loss is compensated by other regional pathways in the direct/indirect pathway, so When motor Sx has developed, pathways outside of SN is also impaired.
      • Gliosis
        • Mild to moderate gliosis accompanied the neuronal loss previously described,
      • also evidence of gliosis (in the absence of detectable neuronal loss) in raphe nucleus, NBM, striatum, GP, dentate nucleus, amygdala, hippocampus, and cerebral cortices

Unmet Needs

Unmet needs:

  • Halting : one and only!,
  • (restore) ↑ DA neurotransmission no L-dopa no LID, no fluctuation
  • (partial) Neuroprotection / (partial) ↑ DA neurotransmission ↓ L-DOPA dose ↓ LID

Vector production

0602Sarah
  • From GGT side: We have ordered some reagents to derive a potency assay. Our reagents are just starting to arrive. We shall get these going as soon as we have everything we need in-house.
  • I have a new direct report, Amany Awad, who shall be leading these efforts together with Xiuxia in Vector Production and Mingfang in Analytics -
  • The rAAV9 vector production is also on-going at UMass.

PINK-1

  • codes for a mitochondrial membrane anchored-kinase protein,

Mutations

  • AR (ie 대부분의 PINK-1 PD 환자는 Biallelic carrier이다. 드물게 heterozygous 도 발견된다.)
  • Several different PINK1 mutations, primarily missense and nonsense mutations, have been identified in PD families. Mostly loss-of-function
  • 60 mutations of different types (missense, nonsense, splicing, frameshift and deletions)

  • missense mutations, the most frequent type, in 47.6%,
  • The most frequent mutation of all was the missense mutation c.1040T>C, resulting in an amino acid change of leucine to proline at position 347

Uncertain Spans

  • In the DMPK SIMOA CSF note, the value after dmpk 는 is visually small; recorded as 50?.
  • The pS65-Ub assays reagent names/catalog strings are small; obvious OCR errors were corrected against the image, but catalog punctuation may need later confirmation.
  • The lower TMQB timing table has one visible row with blank/cut-off time for Current status of asset generation.
  • The PINK-1 section continues below the photo boundary; only visible lines are transcribed here.