Tables of NER proteins

Tables

Table I: Main clinical symptoms of NER deficient syndromes
Clinical symptoms	XP	XP/CS	CS	TTD
Photosensitivity	++	++	+	+
Abnormal pigmentation	++	+	-	-
Skin cancer	++	+	-	-
Neuron degeneration	+/-	+	?	?
Neuron dysmyelination	-	++	++	+
Wizened facies	-	+	+	+
Growth defect	+/-	+	+	+
Hypogonadism	-	+	+	+
Brittle hair and nails	-	-	-	+
Scaling of skin	-	-	-	+
table adapted from [Vermeulen, 1997]

Table II: Roles of NER proteins and residual repair capacity in NER deficient cells
	NER			BER		protein function
	6-4	CPD		TCR	GGR
		TCR	GGR	TCR	GGR
XP-A	-	-	-	+	+	damage recognition
XP-B	-	-	-	+	+	3' - 5' helicase promoter clearance
XP-C	-	+	-	+	+	damage recognition (GGR only)
XP-D	-	-	-	+	+	5' - 3' helicase promoter clearance
XP-E	slow	+	+/-	+	+	damage recognition
XP-F	-	-	+	+	+	5' incision (damage recognition ?)
XP-G	-	-	-	+	+	3' incision enhance BER
CS-A	+	-	+	-	+	TCR/recovery of RNA synthesis
CS-B	+	-	+	-	+	TCR/recovery of RNA synthesis transcript elongation
XPB/CS	-	-	-	(-)	+	same as XP-B
XPD/CS	-	-	-	(-)	+	same as XP-D
XPG/CS	-	-	-	-	-	same as XP-G
TTD - I	+	+	+	+	+	unknown
TTD - II	+	-	-	?	+	same as XPB or XPD
TTD - III	+/-	-	-	?	+	same as XPB or XPD
RPA	-	+	-	+	+	damage recognition

+ = no evidence of repair defect
- = significantly impaired repair capacity
+/- = inconclusive results
? = has not been examined
(-) = proposed (but unsubstantiated) defect

Table III: Evolutionary Conservation of Eukaryotic NER genes
human disorder	CHO group	*s. cerevisiae*	*s. pombe*	other
XPA		Rad 14 (27% id. / 54% sim.)	rhp 14	haywire^DM (66% / 76%) ERCC3^MM (96% / 98%)
XPB	ERCC 3	Rad25 / SSL 2 (54% id.)	Ercc3
XPC		Rad 4 (23 % id.)		XPC^DM (50% / 68%)
XPD	ERCC 2	Rad 3 (52% id. / 73% sim.)	rhp 3 (rad 15) (56% id. / 75% sim.)	XPD^MM (97% / 99%)
XPE (p125)				XPE^DD (44% id.)
XPE (p48)
XPF	ERCC 4	Rad 1 (28% id. / 51% sim.)	rad 16 (33% id. 57% sim.)	MEI-9^DM (40% / 61%)
XPG	ERCC 5	Rad 2 (24% id. / 39% sim.)	rad 13 (33% id.)	XPG^XL (51% / 60%)
CSA	ERCC 8	Rad 28 (23% id. 36% sim.)
CSB	ERCC 6	Rad 26 (44% id. / 64% sim.)
HHR23B		Rad 23 (33% id. / 41% sim.)		MHR23B^MM (88% / 95%)
	ERCC 1	Rad 10 (35% id.)	Swi 10 (39% id.)	C-terminal region of UvrC^EC (38% sim.)
RPA1 (p70)		33% id. / 45% sim.	39% id. / 50% sim.
RPA2 (p34)		30% id. / 38% sim.	34% id. / 44% sim.
RPA3 (p14)		28% id. / 35% sim.	26% id. / 37% sim.
superscripts indicate species: DM = D. melanogaster (fruit fly), MM = M. musculus (mouse), DD = D. discoideum (slime mold), XL = X. laevis (frog) and EC = E. coli.