Table 3 Correlation between experimental validations and nodes in the FA/BRCA BNM

DNA damage

ICL

MMC

FA cells are

[21, 25, 26]

induction

hypersensitive

to ICL inducing

agents

Upstream

FAcore

Non-evaluated

ICL recognition

[14, 16, 17]

FA/BRCA

FANCD2I

proteins

pathway

NUC1

NUC2

DNA repair

ADD

γH2AX,

ICLs are unhooked

[13, 21]

intermediaries

DSB

CA in metaphase

by FA core-recruited

spreads

DNA-endonucleases

that generate a DSB

and an ADD

Downstream

TLS

Non-evaluated

The ADD and DSB

[14, 15, 18, 54]

FA/BRCA

FAHRR

are repaired by TLS

pathway

and FA-dependent

downstream homologous

recombination repair,

respectively. FA cells

accumulate DSBs

Alternative

HRR2

Non-evaluated

FA cells use

[49, 56]

DNA repair

NHEJ

alternative DNA

pathways

repair pathways,

mainly NHEJ

HRR2 is a

criptic repair choice

Checkpoint

ATR

Cell cycle arrest

Upon DNA damage

[27, 28, 31]

ATM

in G2, pCHK1-S341,

normal and FA

p53

p21 gene expression,

cells activate

MYT1, WEE1, p21

the G2/M checkpoint

proteins

Checkpoint

CHKREC

MPM2 mitotic index,

The checkpoint

[83, 84] and this work

recovery

cytokinesis block assay,

is inactivated by

G2/M transcriptional

CHKREC after

program, WIP1, PLK1,

DNA repair

CDC25, Aurora A

FA cells seem to have

proteins

a lower threshold for

CHKREC activation

compared to normal cells