Table 2.

List of lipid-modified ciliary and flagellar proteins discussed in this review.

Protein	Site of Lipid Modification	Lipid Modification Identification Method	Construct Used to Identify Lipid Modification	Cilia Targeting Requirements (CTS and More)	Disease Association/Function	Reference
Fibrocystin	Palmitoylation on three conserved cysteine residues in CTS	Mutation and metabolic labeling	193 residues of C-terminal	18 intracellular aa flanking the transmembrane domain, including conserved cysteines	ARPKD	[31,32]
Arl13b	Palmitoylation in N-terminal region	Mutation and metabolic labeling	Full length protein	Multiple regions in the protein	JS	[19,33]
Rhodopsin	Palmitoylation in C-terminal cytoplasmic region	Enzymatic and chemical cleavage techniques, tandem mass spectrometry, mutations/deletions and knock-out mice	C-terminal fusion protein	VxPx and the FR (in IMCD3)	Inherited retinal degenerative diseases	[34,35,36]
PC1	Palmitoylation in C-terminal region	Metabolic labeling and Biochemical assays	~200-amino-acid C-terminal tail (CTT)	Multiple sites in the coiled-coil motif in the C-terminal tail including VxP motif and multiple cis-acting elements	Mutation cause ADPKD	[37,38]
RFX3	Palmitoylation on a cysteine residue in the dimerization domain	Biochemical assays and mass spectrometry	Full length protein	-	Ciliopathies and metabolic disorders, like diabetes	[39,40]
Hedgehog	Palmitoylation in N-terminal cysteine	Mass spectrometry and metabolic labeling	Full length protein	-	Organ development and cancer	[41]
Wnt	Palmitoylation on a conserved cysteine in N-terminal region	Mass spectrometry, biochemical, enzymatic methods and mutations	Full length protein	-	Wnt signaling involved in animal development including proliferation of stem cells	[42]
Cystin	Myristoylation on glycine 2	Mutations and metabolic labeling	Full length and various truncated mutants	AxEGG	PKD	[27]
NPHP3	Myristoylation at the N-terminus	Metabolic labeling and mutations	Truncated N-terminal fusion proteins	N-terminal CC domain and the myristoylation site	Nephronophthisis	[43]
CIL-7	Myristoylation at the N-terminus	Mutations	Full length protein	Myristoylation motif	PKD	[44]
CePPEF	N-terminal myristoylation and palmitoylation	Metabolic labeling and mutations	Full length protein and N-terminal recombinants	N-terminal region, palmitoylation is particularly important	Calcium regulation	[45]
Calflagin	Myristoylation at glycine 2 and palmitoylation at cysteine 3 in the N-terminal region	Biochemical assays, metabolic labeling and mutations	Full length protein	Palmitoylation	Calcium binding protein	[20]
RP2	Myristoylation at glycine 2 and palmitoylation at cysteine 3 in the N-terminal region	Mutation	Truncated N-terminal fusion proteins	N-terminal dual lipidation-coupled ciliary targeting signal (nlCTS)	X-linked retinitis pigmentosa	[21,46]
INPP5B	Prenylated at its C-terminus CaaX	Mutation and knock downs	Full length protein	Prenylation	Important for retrograde trafficking	[47,48]
RPGR	Prenylated at its C-terminus CaaX	Mutation	Full length and deletion mutants	Two independent ciliary targeting signals: one within the RLD and the other near the C-terminus.	Inherited retinal degenerative diseases	[49]
INPP5E	prenylated at its C-terminus CaaX	Biochemical assays, enzymatic assays and mutations	Full length protein	FDRELYL (not sufficient, require other interactors)	JS and MORM syndrome	[50,51,52,53]